Tetralogy of Fallot (TOF) in Adults
No Results
No Results
processing….
Tetralogy of Fallot (TOF) (pronounced te-tral-uh-jee of Fal-oh), one of the most common congenital heart disorders, comprises right ventricular (RV) outflow tract obstruction (RVOTO) (infundibular stenosis), ventricular septal defect (VSD), aorta dextroposition (overriding aorta), and RV hypertrophy (see the image below). The mortality rate in untreated patients reaches 50% by age 6 years, but in the present era of cardiac surgery, children with simple forms of tetralogy of Fallot enjoy good long-term survival with an excellent quality of life.
However, it is important to understand that corrective surgery for tetralogy of Fallot performed in childhood is not curative surgery. As a result, many children with repaired tetralogy of Fallot survive into adulthood and are seen at cardiology clinics. [1, 2] Some patients who never underwent surgery for mild tetralogy during childhood may present as adults with a variety of symptoms, and other patients who may only have had a palliative procedure (eg, shunt placement) can also present as adults. [3]
The clinical features of tetralogy of Fallot in the adult are directly related to the severity of the anatomic defects and may include the following:
Lack of exercise endurance
Palpitations
Gradual decline in bodily functions
Infants, however, often display the following:
Difficulty with feeding
Failure to thrive
Episodes of bluish pale skin during crying or feeding (ie, “Tet” spells)
Exertional dyspnea, usually worsening with age
Physical findings in adults include the following:
Exertional dyspnea
Syncope
Palpitations
Evidence of right-sided heart failure (eg, elevated jugular venous pressure [JVP], ascites, peripheral edema, hepatomegaly)
Large “A wave” in JVP tracings
In individuals with pulmonary valve insufficiency: Low-pitched, short diastolic murmur; may have an extra heart sound (ejection click); may have a murmur
RV dysfunction, arrhythmias
See Presentation for more detail.
In adult patients, the following laboratory studies may be helpful:
Complete blood cell count: A mild anemia may be present; polycythemia is rare in the absence of cyanosis.
Coagulation profile: May be abnormal in patients with cyanosis and bleeding
Arterial blood gas (including serum lactate, base excess, oxygen partial pressure [PO2]): The patient’s oxygenation status, serum lactate levels, and base excess value appear to be prognostic markers for mortality in those undergoing surgical repair of tetralogy of Fallot. [4]
Blood cultures: Obtain blood cultures in febrile patients to rule out endocarditis or sepsis.
Imaging studies include the following:
Chest radiographs
Echocardiography
Magnetic resonance imaging (MRI)
Chest radiographs have the following attributes:
May be normal or depict cardiomegaly and prominent RV shadowing
Although the classic boot-shaped heart (coeur en sabot) is the hallmark of the disorder in infants, this shape of the heart may not be seen in adult patients.
Echocardiography has the following attributes:
Color-flow Doppler echocardiography: Assesses overall cardiac function and the status of the valves, and for the presence of any residual VSD, ductus arterosus, or atrial septal defect
Allows evaluation of the valvular anatomy but is unable to visualize the coronary anatomy in adults
Reveals the grade and severity of any RVOT obstruction
MRI has the following attributes:
Gold standard for assessing RV function and size, and for quantifying the pulmonary regurgitant volume
Maps the velocity of pulmonary regurgitation and provides good delineation of the aorta size and assessment of the pulmonary arteries and its branches, the status of the RVOT, and the presence of VSDs, and/or RV hypertrophy [5]
Can also be used to measure intracardiac pressures, gradients, and blood flows
Cardiac catheterization is useful in any of the following settings:
When the anatomy cannot be completely defined by echocardiography
When disease in the pulmonary arteries is a concern
When pulmonary vascular hypertension is possible
Cardiac catheterization allows the following:
Assessment of the pulmonary annulus size and pulmonary arteries
Assessment of the severity of RVOTO
Location of the position and size of the VSD
Ruling out possible coronary artery anomalies
See Workup for more detail. See also the Guidelines section for the 2018 American Heart Association/American College of Cardiology (AHA/ACC) recommendations for the management of adults with tetralogy of Fallot.
For adults with acute cyanosis, place them in a knee-chest position. In addition, provide/administer the following:
Oxygen
IV morphine
IV propranolol for severe cases [6]
Most symptomatic adults with tetralogy of Fallot require some type of surgical intervention. Pulmonary valve replacement is the most common procedure performed, usually under cardiopulmonary bypass.
Factors that increase the risk for surgery in adult patients with tetralogy of Fallot include the following:
Cardiogenic shock
Poor RV function
Diminutive pulmonary arteries
Other major associated anomalies, such as tricuspid atresia or an anomalous coronary artery
Multiple previous surgeries
Advanced age
Severe annular hypoplasia
High peak RV–to–left ventricular pressure ratio
Multiple ventricular septal defects (VSDs)
Right-sided heart failure
See Treatment and Medication for more detail, as well as the Guidelines section for the 2018 American Heart Association/American College of Cardiology (AHA/ACC) recommendations for the management of adults with tetralogy of Fallot.
Tetralogy of Fallot (TOF) (pronounced te-tral-uh-jee of Fal-oh) is one of the most common congenital heart disorders (CHDs). This condition is classified as a cyanotic heart disorder, because tetralogy of Fallot results in an inadequate flow of blood to the lungs for oxygenation (right-to-left shunt) (see the following image). Patients with tetralogy of Fallot initially present with cyanosis shortly after birth, thereby attracting early medical attention. In most cases, the cyanosis does not present for a few weeks or months after birth; however, infants born with transposition of the great vessels usually present with cyanosis immediately after birth.
The four features typical of tetralogy of Fallot include right ventricular (RV) outflow tract obstruction (RVOTO) (infundibular stenosis), ventricular septal defect (VSD), aorta dextroposition (overriding aorta), and right ventricular hypertrophy. Occasionally, a few children also have an atrial septal defect (ASD), which makes up the pentad of Fallot. The basic pathology of tetralogy is due to the underdevelopment of the RV infundibulum, which results in an anterior-leftward malalignment of the infundibular septum. This malalignment determines the degree of RVOTO.
The clinical features of tetralogy of Fallot are generally typical, and a preliminary clinical diagnosis can almost always be made. Because most infants with this disorder require surgery, it is fortunate that the availability of cardiopulmonary bypass (CPB), cardioplegia, and surgical techniques is now well established. Most surgical series report excellent clinical results with low morbidity and mortality rates.
The first surgery to repair tetralogy of Fallot consisted of placement of a shunt to relieve the cyanosis. Primary repair is currently recommended within the first 12 months of life; in general, excellent results are obtained at most centers. Ever since primary repair of tetralogy of Fallot became the standard of care nearly 30 years ago, more adult patients with repaired tetralogy of fallot in early childhood are living longer, [1, 2] and they are being seen at many cardiac clinics. Some patients who never underwent surgery for mild tetralogy during childhood may present as adults with a variety of symptoms, and other patients who may only have had a palliative procedure (eg, shunt placement) can also present as adults. [3]
It is important to understand that corrective surgery for tetralogy of Fallot performed in childhood is not curative surgery. Rather, this procedure only corrects the anatomic defects—without changing the progression of the disease or addressing the consequences of using a patch. As a result, these individuals develop new symptoms and eventually present to the cardiology clinic with diverse symptoms. Thus, most pediatric surgeons consider the initial surgery for tetralogy of Fallot to be long-term palliation, not definitive surgery.
With advancing age, children with tetralogy of Fallot who had corrective surgery are usually asymptomatic for the first decade after the initial procedure. Thereafter, they develop adverse myocardial problems, the majority of which involve varying degrees of pulmonary valve insufficiency, which, in turn, can lead to RV overload and RV distention and failure. [7]
RV enlargement also leads to the development of atrial and ventricular arrhythmias, which, if not recognized early, are a common cause of high morbidity and mortality. An estimated one third of adults with tetralogy of Fallot experience atrial and ventricular arrhythmias over a period of 2-3 decades. The incidence of sudden death ranges from 5% to 7% in patients who develop these arrhythmias.
Another concern regarding patients with tetralogy of Fallot is that many are lost to follow-up as they become adults, and thus the opportunity to manage complications before they become irreversible can be missed. Several surveys indicate that many children with repaired tetralogy of Fallot feel fine after surgery, believe that they are cured, and then stop seeing their cardiologist. In other cases, the primary care provider is unaware of the need to follow these patients, owing to the erroneous belief that surgery is curative.
See also Tetralogy of Fallot With Pulmonary Stenosis, Tetralogy of Fallot With Pulmonary Atresia, and Tetralogy of Fallot With Absent Pulmonary Valve.
Louis Arthur Fallot, after whom the name tetralogy of Fallot is derived, was not the first person to recognize the condition. Stensen first described it in 1672; however, it was Fallot who first accurately described the clinical and complete pathologic features of the defects.
Although the disorder was clinically diagnosed much earlier, no treatment was available until the 1940s. Cardiologist Helen Taussig recognized that cyanosis progressed and inevitably led to death in infants with tetralogy of Fallot. She postulated that the cyanosis was due to inadequate pulmonary blood flow. Her collaboration with Alfred Blalock led to the first type of palliation for these infants. In 1944, Blalock operated on an infant with tetralogy of Fallot and created the first Blalock-Taussig shunt between the subclavian artery and the pulmonary artery (see the image below).
The pioneering Blalock-Taussig shunt surgical technique opened a new era in neonatal cardiac surgery. Development of the Potts shunt (from the descending aorta to the left pulmonary artery), the Glenn shunt (from the superior vena cava to the right pulmonary artery), and the Waterston shunt (from the ascending aorta to the right pulmonary artery) followed.
Scott performed the first open correction in 1954. Less than 6 months later, Lillehei performed the first successful open repair for tetralogy of Fallot using controlled cross-circulation, with another patient serving as oxygenator and blood reservoir. The following year, with the advent of cardiopulmonary bypass by Gibbons, another historic era of cardiac surgery was established. Since then, numerous advances in surgical technique and myocardial preservation have evolved in the treatment of tetralogy of Fallot.
The classic anatomic features of adult patients with tetralogy of Fallot (TOF) are no different from those seen in pediatric patients, such as the following [8] :
The aorta in patients with tetralogy of Fallot is hugely dilated and overrides the diminutive pulmonary artery. In about 25% of cases, the aorta arises from a right-sided aortic arch. Because of this feature and overriding of the septum, about 50% of the blood flow from the aorta arises from the RV via the large perimembranous VSD.
In the majority of adults with tetralogy of Fallot, there is resistance to RV emptying. The obstruction is caused by the anterior displacement and rotation of the infundibular septum which narrows the outflow tract. The infundibular obstruction may be associated with pulmonary valve stenosis or atresia, which results in further outflow tract obstruction.
The size of the pulmonary arteries in tetralogy of Fallot vary in size and distribution. In most cases, the vessels are hypoplastic but can be atretic in severe cases. An almost universally common feature of tetralogy of Fallot is reduced blood flow in the pulmonary arteries and, hence cyanosis. There are also reported cases of an absent left pulmonary artery. In close to three quarters of patients with TOF, there is pulmonary valve stenosis, which is often due to leaflet tethering rather than commissural fusion. In virtually all such cases, the pulmonary annulus is narrowed.
Associated heart defects are very common in tetralogy of Fallot. Some patients may have an atrial septal defect, often referred to as part of the pentad of Fallot. Other common defects include a patent ductus arteriosus, muscular VSD, atrioventricular septal defects, anomalous coronary arteries, anomalous pulmonary venous return, absent pulmonary valve, aortic incompetence, and aortopulmonary window. [9, 10]
The cause(s) of most congenital heart diseases (CHDs) are unknown, although genetic studies suggest a multifactorial etiology. Methylene tetrahydrofolate reductase (MTHFR) gene polymorphism may be a susceptibility gene for tetralogy of Fallot (TOF). [11, 12] More recently, it appears that VEGF genetic polymorphisms, -2578C>A and -634C>G, may be associated with an increased risk for tetralogy of Fallot, whereas the risk is potentially reduced with 936C>T polymorphism. [13]
Prenatal factors associated with a higher incidence of tetralogy of Fallot include maternal rubella (or other viral illnesses) during pregnancy, poor prenatal nutrition, maternal alcohol use, maternal age older than 40 years, maternal phenylketonuria (PKU) birth defects, and diabetes. Children with Down syndrome also have a higher incidence of tetralogy of Fallot, as do infants with fetal hydantoin syndrome or fetal carbamazepine syndrome.
In addition, as one of the conotruncal malformations, tetralogy of Fallot can be associated with a spectrum of lesions known as CATCH 22 (cardiac defects, abnormal facies, thymic hypoplasia, cleft palate, hypocalcemia). Cytogenetic analysis may demonstrate deletions of a segment of chromosome band 22q11 (DiGeorge critical region). Ablation of cells of the neural crest has been shown to reproduce conotruncal malformations.
These abnormalities are associated with the DiGeorge syndrome and branchial arch abnormalities.
The hemodynamics of tetralogy of Fallot depend on the degree of right ventricular (RV) outflow tract obstruction (RVOTO). The ventricular septal defect (VSD) is usually nonrestrictive, and the RV and left ventricular (LV) pressures are equalized. If the obstruction is severe, the intracardiac shunt is from right to left, and pulmonary blood flow may be markedly diminished. In this instance, blood flow may depend on the patent ductus arteriosus (PDA) or bronchial collaterals.
Previously, clinicians often ordered routine echocardiograms in adult patients with tetralogy of Fallot who were followed in the clinic following surgery in infancy. At that time, it was felt that, as in cases of tricuspid regurgitation, pulmonary valve insufficiency was a relatively benign and inconsequential observed entity. Because most affected patients were not symptomatic, they were never treated. However, observation of these patients over time revealed that they began to develop severe RV dysfunction and arrhythmias. The RV dilatation from the pulmonary valve insufficiency is associated with fibrosis and severe myocardial damage, which then often lead to a decrease in exercise endurance, and the majority of patients soon develop ventricular arrhythmias. [14]
Initially most patients with chronic RV function are asymptomatic, but as the compensatory mechanisms fail and the ejection fraction decreases, symptoms start to appear. If the condition is not treated at this stage, the RV dysfunction is irreversible. The arrhythmias occur as a result of progressive dilatation and stretching of the right atrium and RV.
Atrial arrhythmias of clinical significance are chiefly of the reentry type, but they may also include atrial tachycardia and atrial fibrillation. These atrial arrhythmias occur in 10-35% of patients with repaired tetralogy of Fallot. [15]
Ventricular arrhythmias and sudden death are also known to occur in those with repaired tetralogy of Fallot. Case studies have revealed that patients who experience sudden death often have moderate to severe pulmonary valve insufficiency at the time of death. Ventricular arrhythmias tend to be less common in patients with mild pulmonary valve insufficiency.
Overall, the risk of late sudden death is many times greater in patients who survive tetralogy of Fallot surgery than in their age-matched counterparts. [16]
Tetralogy of Fallot (TOF) represents approximately 7%-10% of congenital heart diseases (CHDs), [2] and it is the most common cyanotic CHD, with 0.23-0.63 cases per 1,000 births. [1] This disorder accounts for one third of all CHD in patients younger than 15 years; in adults, tetralogy of Fallot has an estimated prevalence of 1 in 3,500 to 1 in 4,300 people. [2]
In most cases, tetralogy of Fallot is sporadic and nonfamilial. The incidence in siblings of affected parents is 1-5%, and it occurs more commonly in males than in females. The disorder is associated with extracardiac anomalies such as cleft lip and palate, hypospadias, and skeletal and craniofacial abnormalities. Genetic studies indicate that in some patients with tetralogy of Fallot, there may be 22q11.2 deletion and other submicroscopic copy number alterations. [17]
Adult patients with tetralogy of Fallot currently represent a very large group of patients who underwent congenital heart surgery in early life. Although the exact number of these adults is not known, because many are lost to follow-up or have never been followed, it is estimated that over two thirds of affected children who undergo repair of tetralogy of Fallot in early childhood will reach adulthood, with one study showing 94% survival rate of 168 patients aged 16 years and older who underwent simple repair. [16] For individuals born with this condition, the 30-year survival is above 75%, provided these individuals have been clinically followed. Limited data to date reveal that adult tetralogy of Fallot is equally common in both sexes.
The majority of adult patients with repaired tetralogy of Fallot present after the second or third decade of life. Males and females appear to be equally affected with symptoms as they age. [18]
In the present era of cardiac surgery, children with simple forms of tetralogy of Fallot (TOF) enjoy good long-term survival with an excellent quality of life. Late outcome data suggest that most survivors are in New York Heart Association (NYHA) classification I, although maximal exercise capability is reduced in some.
About 75% infants who undergo repair during infancy will survive to reach their second to third decade of life without major consequences. However, after the first two decades of life, symptoms start to appear due to pulmonary valve regurgitation. By the fourth decade of life, most survivors are symptomatic.
Adult patients with tetralogy of Fallot who undergo surgery again are usually symptom free for 10-15 years, but by the time they reach their fifth decade, even these patients begin to have symptoms. [16] Although the second surgery reduces the rate of death, the majority of these individuals have a shorter lifespan than age-matched control subjects without a history of congenital heart disease. Adults with recalcitrant arrhythmias and right heart failure have the worst prognosis.
Sudden death from ventricular arrhythmias has been reported in 1-5% of patients at a later stage in life, and the cause remains unknown. It has been suspected that ventricular dysfunction may be the cause. One study found left ventricular longitudinal dysfunction to be associated with a greater risk of developing life-threatening arrhythmias. [19] Continued cardiac monitoring into adult life is necessary. For some time, it has been suspected that certain children may have inherited a predispostion to developing long QT syndrome. A 2012 study by Chiu confirmed this suspicion. [20]
Most individuals who survive to age 30 years develop congestive heart failure (CHF), although individuals whose shunts produce minimal hemodynamic compromise have been noted, albeit rarely, and these individuals achieve a normal life span. Survival of patients into their 80s have been reported. Due to advanced surgical techniques, a 40% reduction in deaths associated with tetralogy of Fallot was noted from 1979 to 2005. [18]
Adults with repaired tetralogy of Fallot during infancy can present with the following complications later in life:
Right heart failure
Atrial and ventricular arrhythmias
Sudden death
Endocarditis
Septic central emboli
See Surgical Complications in patients with repaired tetralogy of Fallot.
Refaat MM, Ballout J, Mansour M. Ablation of atrial fibrillation in patients with congenital heart disease. Arrhythm Electrophysiol Rev. 2017 Dec. 6 (4):191-4. [Medline].
Maury P, Sacher F, Rollin A, et al. Ventricular arrhythmias and sudden death in tetralogy of Fallot. Arch Cardiovasc Dis. 2017 May. 110 (5):354-62. [Medline].
Fraser CD, Bacha EA, Comas J, Sano S, Sarris GE, Tsang VT. Tetralogy of Fallot. Semin Thorac Cardiovasc Surg. 2015 Summer. 27 (2):189-204. [Medline].
Bhardwaj V, Kapoor PM, Irpachi K, Ladha S, Chowdhury UK. Basic arterial blood gas biomarkers as a predictor of mortality in tetralogy of Fallot patients. Ann Card Anaesth. 2017 Jan-Mar. 20 (1):67-71. [Medline].
Boechat MI, Ratib O, Williams PL, Gomes AS, Child JS, Allada V. Cardiac MR imaging and MR angiography for assessment of complex tetralogy of Fallot and pulmonary atresia. Radiographics. 2005 Nov-Dec. 25(6):1535-46. [Medline].
Gabriele B, Maurizio B, Marco B. Does pharmacological therapy still play a role in preventing sudden death in surgically treated tetralogy of Fallot?. Mini Rev Med Chem. 2018. 18 (6):490-4. [Medline].
Faccini A, Micheletti A, Negura DG, et al. Heart failure in grown-up congenital heart disease. Minerva Cardioangiol. 2018 Jun. 66 (3):329-36. [Medline].
Anderson RH, Weinberg PM. The clinical anatomy of tetralogy of Fallot. Cardiol Young. 2005 Feb. 15 Suppl 1:38-47. [Medline].
Sarris GE. Tetralogy surgery – back to Baltimore 70 years later: Melbourne Heritage and Group tribute to Juan Comas. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2017 Jan. 20:84-8. [Medline].
Karl TR, Stocker C. Tetralogy of fallot and its variants. Pediatr Crit Care Med. 2016 Aug. 17 (8 suppl 1):S330-6. [Medline].
Marinho C, Alho I, Guerra A, Rego C, Areias J, Bicho M. The methylenetetrahydrofolate reductase gene variant (C677T) as a susceptibility gene for tetralogy of Fallot. Rev Port Cardiol. 2009 Jul-Aug. 28(7-8):809-12. [Medline].
Lee CN, Su YN, Cheng WF, et al. Association of the C677T methylenetetrahydrofolate reductase mutation with congenital heart diseases. Acta Obstet Gynecol Scand. 2005 Dec. 84(12):1134-40. [Medline].
Li X, Liu CL, Li XX, Li QC, Ma LM, Liu GL. VEGF gene polymorphisms are associated with risk of tetralogy of Fallot. Med Sci Monit. 2015 Nov 12. 21:3474-82. [Medline].
Anabtawi A, Mondragon J, Dodendorf D, Laskey WK. Late-stage left ventricular dysfunction in adult survivors of tetralogy of Fallot repair in childhood. Open Heart. 2017. 4 (2):e000690. [Medline].
Wu MH, Lu CW, Chen HC, Kao FY, Huang SK. Adult congenital heart disease in a nationwide population 2000-2014: epidemiological trends, arrhythmia, and standardized mortality ratio. J Am Heart Assoc. 2018 Feb 8. 7 (4):[Medline].
Dennis M, Moore B, Kotchetkova I, Pressley L, Cordina R, Celermajer DS. Adults with repaired tetralogy: low mortality but high morbidity up to middle age. Open Heart. 2017. 4 (1):e000564. [Medline].
Rauch R, Hofbeck M, Zweier C, et al. Comprehensive genotype-phenotype analysis in 230 patients with tetralogy of Fallot. J Med Genet. 2010 May. 47(5):321-31. [Medline].
Pillutla P, Shetty KD, Foster E. Mortality associated with adult congenital heart disease: trends in the US population from 1979 to 2005. Am Heart J. 2009 Nov. 158(5):874-9. [Medline].
Diller GP, Kempny A, Liodakis E, et al. Left ventricular longitudinal function predicts life-threatening ventricular arrhythmia and death in adults with repaired tetralogy of fallot. Circulation. 2012 May 22. 125(20):2440-6. [Medline].
Chiu SN, Wu MH, Su MJ, et al. Coexisting mutations/polymorphisms of the long QT syndrome genes in patients with repaired Tetralogy of Fallot are associated with the risks of life-threatening events. Hum Genet. 2012 Aug. 131(8):1295-304. [Medline].
Dluzniewska N, Podolec P, Miszalski-Jamka T, et al. Effect of ventricular function and volumes on exercise capacity in adults with repaired Tetralogy of Fallot. Indian Heart J. 2018 Jan – Feb. 70 (1):87-92. [Medline].
Vehmeijer JT, Koyak Z, Bokma JP, et al. Sudden cardiac death in adults with congenital heart disease: does QRS-complex fragmentation discriminate in structurally abnormal hearts?. Europace. 2018 Jun 1. 20 (FI1):f122-8. [Medline].
Gaydos SS, Varga-Szemes A, Judd RN, Suranyi P, Gregg D. Imaging in adult congenital heart disease. J Thorac Imaging. 2017 Jul. 32 (4):205-16. [Medline].
Bokma JP, de Wilde KC, Vliegen HW, et al. Value of cardiovascular magnetic resonance imaging in noninvasive risk stratification in tetralogy of Fallot. JAMA Cardiol. 2017 Jun 1. 2 (6):678-83. [Medline].
Scalone G, Gomez-Monterrosas O, Fiszer R, Szkutnik M, Galeczka M, Bialkowski J. Combined strategy of Waterston shunt percutaneous occlusion and medical treatment with sildenafil for management of pulmonary hypertension in an adult patient with corrected tetralogy of Fallot. Postepy Kardiol Interwencyjnej. 2017. 13 (3):277-8. [Medline].
McRae ME, Coleman B, Atz TW, Kelechi TJ. Patient outcomes after transcatheter and surgical pulmonary valve replacement for pulmonary regurgitation in patients with repaired tetralogy of Fallot: A quasi-meta-analysis. Eur J Cardiovasc Nurs. 2017 Aug. 16 (6):539-53. [Medline].
Bhagra CJ, Hickey EJ, Van De Bruaene A, Roche SL, Horlick EM, Wald RM. Pulmonary valve procedures late after repair of tetralogy of Fallot: current perspectives and contemporary approaches to management. Can J Cardiol. 2017 Sep. 33 (9):1138-49. [Medline].
Pragt H, van Melle JP, Javadikasgari H, et al. Mechanical valves in the pulmonary position: an international retrospective analysis. J Thorac Cardiovasc Surg. 2017 Oct. 154 (4):1371-8.e1. [Medline].
Therrien J, Marx GR, Gatzoulis MA. Late problems in tetralogy of Fallot–recognition, management, and prevention. Cardiol Clin. 2002 Aug. 20 (3):395-404. [Medline].
Ezzat VA, Ryan MJ, O’Leary J, et al. Radiofrequency ablation of atrial tachyarrhythmias in adults with tetralogy of Fallot – predictors of success and outcome. Cardiol Young. 2017 Mar. 27 (2):284-93. [Medline].
Davidson J, Tong S, Hancock H, Hauck A, da Cruz E, Kaufman J. Prospective validation of the vasoactive-inotropic score and correlation to short-term outcomes in neonates and infants after cardiothoracic surgery. Intensive Care Med. 2012 Jul. 38(7):1184-90. [Medline].
Menting ME, van den Bosch AE, McGhie JS, et al. Assessment of ventricular function in adults with repaired Tetralogy of Fallot using myocardial deformation imaging. Eur Heart J Cardiovasc Imaging. 2015 Dec. 16(12):1347-57. [Medline].
Lindsey CW, Parks WJ, Kogon BE, Sallee D 3rd, Mahle WT. Pulmonary valve replacement after tetralogy of Fallot repair in preadolescent patients. Ann Thorac Surg. 2010 Jan. 89(1):147-51. [Medline].
Bokma JP, Geva T, Sleeper LA, et al. A propensity score-adjusted analysis of clinical outcomes after pulmonary valve replacement in tetralogy of Fallot. Heart. 2018 May. 104 (9):738-44. [Medline].
Heng EL, Gatzoulis MA, Uebing A, et al. Immediate and midterm cardiac remodeling after surgical pulmonary Vvalve replacement in adults with repaired tetralogy of Fallot: a prospective cardiovascular magnetic resonance and clinical study. Circulation. 2017 Oct 31. 136 (18):1703-13. [Medline].
Mitropoulos FM, Kanakis MA, Ntellos C, et al. Pulmonary valve replacement in patients with corrected tetralogy of Fallot. J Cardiovasc Thorac Res. 2017. 9 (2):71-7. [Medline].
Caruana M, Grech V. A first population-based long-term outcome study in adults with repaired tetralogy of Fallot in Malta. Congenit Heart Dis. 2017 May. 12 (3):301-8. [Medline].
Kanter KR, Kogon BE, Kirshbom PM, Carlock PR. Symptomatic neonatal tetralogy of Fallot: repair or shunt?. Ann Thorac Surg. 2010 Mar. 89(3):858-63. [Medline].
Tsang FH, Li X, Cheung YF, Chau KT, Cheng LC. Pulmonary valve replacement after surgical repair of tetralogy of Fallot. Hong Kong Med J. 2010 Feb. 16(1):26-30. [Medline].
Alkashkari W, Alsubei A, Hijazi ZM. Transcatheter pulmonary valve replacement: current state of art. Curr Cardiol Rep. 2018 Mar 15. 20 (4):27. [Medline].
Jones MI, Qureshi SA. Recent advances in transcatheter management of pulmonary regurgitation after surgical repair of tetralogy of Fallot. F1000Res. 2018. 7:[Medline].
Plessis J, Hascoet S, Baruteau A, et al. Edwards SAPIEN transcatheter pulmonary valve implantation: results from a French registry. JACC Cardiovasc Interv. 2018 Oct 8. 11 (19):1909-16. [Medline].
[Guideline] Cotts TB. 2018 AHA/ACC guideline for adults with congenital heart disease. American College of Cardiology. Available at https://www.acc.org/latest-in-cardiology/ten-points-to-remember/2018/08/13/16/26/2018-aha-acc-guideline-for-the-management-of-achd. August 16, 2018; Accessed: October 25, 2018.
Senzaki H, Ishido H, Iwamoto Y, et al. Sedation of hypercyanotic spells in a neonate with tetralogy of Fallot using dexmedetomidine. J Pediatr (Rio J). 2008 Jul-Aug. 84(4):377-80. [Medline].
Rodan L, McCrindle BW, Manlhiot C, et al. Stroke recurrence in children with congenital heart disease. Ann Neurol. 2012 Jul. 72(1):103-11. [Medline].
Tanel RE. ECGs in the ED. Pediatr Emerg Care. 2007 Jun. 23(6):428-9. [Medline].
[Guideline] Witte KK, Pepper CB, Cowan JC, Thomson JD, English KM, Blackburn ME. Implantable cardioverter-defibrillator therapy in adult patients with tetralogy of Fallot. Europace. 2008 Aug. 10(8):926-30. [Medline].
Khairy P, Harris L, Landzberg MJ, et al. Implantable cardioverter-defibrillators in tetralogy of Fallot. Circulation. 2008 Jan 22. 117(3):363-70. [Medline].
Chrysostomou C, Sanchez De Toledo J, Avolio T, et al. Dexmedetomidine use in a pediatric cardiac intensive care unit: can we use it in infants after cardiac surgery?. Pediatr Crit Care Med. 2009 Nov. 10(6):654-60. [Medline].
Shepard SM, Tejman-Yarden S, Khanna S, Davis CK, Batra AS. Dexmedetomidine-related atrial standstill and loss of capture in a pediatric patient after congenital heart surgery. Crit Care Med. 2011 Jan. 39(1):187-9. [Medline].
Al Habib HF, Jacobs JP, Mavroudis C, et al. Contemporary patterns of management of tetralogy of Fallot: data from the Society of Thoracic Surgeons Database. Ann Thorac Surg. 2010 Sep. 90(3):813-9; discussion 819-20. [Medline].
Gustafson RA, Murray GF, Warden HE, Hill RC, Rozar GE Jr. Early primary repair of tetralogy of Fallot. Ann Thorac Surg. 1988 Mar. 45(3):235-41. [Medline].
Park CS, Lee JR, Lim HG, Kim WH, Kim YJ. The long-term result of total repair for tetralogy of Fallot. Eur J Cardiothorac Surg. 2010 Sep. 38(3):311-7. [Medline].
Robinson JD, Rathod RH, Brown DW, et al. The evolving role of intraoperative balloon pulmonary valvuloplasty in valve-sparing repair of tetralogy of Fallot. J Thorac Cardiovasc Surg. 2011 Dec. 142(6):1367-73. [Medline].
Pacifico AD, Kirklin JK, Colvin EV, McConnell ME, Kirklin JW. Transatrial-transpulmonary repair of tetralogy of Fallot. Semin Thorac Cardiovasc Surg. 1990 Jan. 2(1):76-82. [Medline].
Yao Q, Hu XH, Shen QL, et al. Differential effect of the ratio of right ventricular volume to left ventricular volume in children with repaired tetralogy of Fallot. Cardiology. 2016. 133(3):135-40. [Medline].
Aboulhosn J, Child JS. Management after childhood repair of tetralogy of fallot. Curr Treat Options Cardiovasc Med. 2006 Dec. 8(6):474-83. [Medline].
Devore GR, Polanko B. Tomographic ultrasound imaging of the fetal heart: a new technique for identifying normal and abnormal cardiac anatomy. J Ultrasound Med. 2005 Dec. 24(12):1685-96. [Medline].
Duro RP, Moura C, Leite-Moreira A. Anatomophysiologic basis of tetralogy of Fallot and its clinical implications. Rev Port Cardiol. 2010 Apr. 29(4):591-630. [Medline].
Fox D, Devendra GP, Hart SA, Krasuski RA. When ‘blue babies’ grow up: What you need to know about tetralogy of Fallot. Cleve Clin J Med. 2010 Nov. 77 (11):821-8. [Medline].
He GW. Current strategy of repair of tetralogy of Fallot in children and adults: emphasis on a new technique to create a monocusp-patch for reconstruction of the right ventricular outflow tract. J Card Surg. 2008 Nov-Dec. 23 (6):592-9. [Medline].
Horer J, Friebe J, Schreiber C, et al. Correction of tetralogy of Fallot and of pulmonary atresia with ventricular septal defect in adults. Ann Thorac Surg. 2005 Dec. 80 (6):2285-91. [Medline].
Hovels-Gurich HH, Konrad K, Skorzenski D, et al. Long-term behavior and quality of life after corrective cardiac surgery in infancy for tetralogy of Fallot or ventricular septal defect. Pediatr Cardiol. 2007 Sep-Oct. 28 (5):346-54. [Medline].
Kantorova A, Zbieranek K, Sauer H, Lilje C, Haun C, Hraska V. Primary early correction of tetralogy of Fallot irrespective of age. Cardiol Young. 2008 Apr. 18 (2):153-7. [Medline].
Laidman J. Fluconazole linked to increased risk for rare birth defect. Medscape Medical News from WebMD. August 28, 2013. Available at http://www.medscape.com/viewarticle/810149. Accessed: September 5, 2013.
Miatton M, De Wolf D, François K, Thiery E, Vingerhoets G. Intellectual, neuropsychological, and behavioral functioning in children with tetralogy of Fallot. J Thorac Cardiovasc Surg. 2007 Feb. 133 (2):449-55. [Medline].
Molgaard-Nielsen D, Pasternak B, Hviid A. Use of oral fluconazole during pregnancy and the risk of birth defects. N Engl J Med. 2013 Aug 29. 369 (9):830-9. [Medline].
Patel CR, Agamanolis DP, Stewart JW. Prenatal diagnosis of tetralogy of Fallot with obstructed supracardiac totally anomalous pulmonary venous connection. Cardiol Young. 2005 Dec. 15 (6):656-9. [Medline].
Redington AN. Determinants and assessment of pulmonary regurgitation in tetralogy of Fallot: practice and pitfalls. Cardiol Clin. 2006 Nov. 24 (4):631-9, vii. [Medline].
Sakamoto T, Nagase Y, Hasegawa H, Shin’oka T, Tomimatsu H, Kurosawa H. One-stage intracardiac repair in combination with external stenting of the trachea and right bronchus for tetralogy of Fallot with an absent pulmonary valve and tracheobronchomalacia. J Thorac Cardiovasc Surg. 2005 Dec. 130 (6):1717-8. [Medline].
Srivastava S, Salem Y, Chatterjee S, et al. Echocardiographic myocardial deformation evaluation of right ventricular function in comparison with CMRI in repaired tetralogy of Fallot: a cross-sectional and longitudinal validation study. Echocardiography. 2013 Feb. 30 (2):196-202. [Medline].
Starr JP. Tetralogy of Fallot: yesterday and today. World J Surg. 2010 Apr. 34 (4):658-68. [Medline].
Aguayo-Gomez A, Arteaga-Vazquez J, Svyryd Y, et al. Identification of copy number variations in isolated tetralogy of Fallot. Pediatr Cardiol. 2015 Dec. 36 (8):1642-6. [Medline].
Shahanavaz S, Qureshi AM, Levi DS, et al. Transcatheter pulmonary valve replacement with the Melody valve in small diameter expandable right ventricular outflow tract conduits. JACC Cardiovasc Interv. 2018 Mar 26. 11 (6):554-64. [Medline].
Naidu P, Grigg L, Zentner D. Mortality in adults with congenital heart disease. Int J Cardiol. 2017 Oct 15. 245:125-30. [Medline].
Apostolopoulou SC, Vagenakis G, Rammos S. Pulmonary vasodilator therapy in tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals: case series and review of literature. Cardiol Young. 2017 Nov. 27 (9):1861-4. [Medline].
[Guideline] Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2018 Aug 10. [Medline]. [Full Text].
Kwak JG, Kim WH, Kim ER, Lim JH, Min J. One-year follow-up after tetralogy of Fallot total repair preserving pulmonary valve and avoiding right ventriculotomy. Circ J. 2018 Oct 5. [Medline].
Shabir Bhimji, MD, PhD Cardiothoracic and Vascular Surgeon, Saudi Arabia and Middle East Hospitals
Shabir Bhimji, MD, PhD is a member of the following medical societies: American Cancer Society, American College of Chest Physicians, American Lung Association, Texas Medical Association
Disclosure: Nothing to disclose.
Mary C Mancini, MD, PhD, MMM Surgeon-in-Chief and Director of Cardiothoracic Surgery, Christus Highland
Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Phi Beta Kappa, Society of Thoracic Surgeons
Disclosure: Nothing to disclose.
Yasmine S Ali, MD, FACC, FACP, MSCI President, Nashville Preventive Cardiology, PLLC; Assistant Clinical Professor of Medicine, Vanderbilt University School of Medicine
Yasmine S Ali, MD, FACC, FACP, MSCI is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, National Lipid Association, Tennessee Medical Association
Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: MCG Health, LLC.
David FM Brown, MD Associate Professor, Division of Emergency Medicine, Harvard Medical School; Vice Chair, Department of Emergency Medicine, Massachusetts General Hospital
David FM Brown, MD is a member of the following medical societies: American College of Emergency Physicians and Society for Academic Emergency Medicine
Disclosure: lippincott Royalty textbook royalty; wiley Royalty textbook royalty
David A Donson, MD Assistant Medical Director, Department of Emergency Medicine, Maimonides Medical Center
David A Donson, MD is a member of the following medical societies: American College of Emergency Physicians and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.
Theodore J Gaeta, DO, MPH, FACEP Clinical Associate Professor, Department of Emergency Medicine, Weill Cornell Medical College; Vice Chairman and Program Director of Emergency Medicine Residency Program, Department of Emergency Medicine, New York Methodist Hospital; Academic Chair, Adjunct Professor, Department of Emergency Medicine, St George’s University School of Medicine
Theodore J Gaeta, DO, MPH, FACEP is a member of the following medical societies: Alliance for Clinical Education, American College of Emergency Physicians, Clerkship Directors in Emergency Medicine, Council of Emergency Medicine Residency Directors, New York Academy of Medicine, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.
Ronald J Oudiz, MD, FACP, FACC, FCCP Professor of Medicine, University of California, Los Angeles, David Geffen School of Medicine; Director, Liu Center for Pulmonary Hypertension, Division of Cardiology, LA Biomedical Research Institute at Harbor-UCLA Medical Center
Ronald J Oudiz, MD, FACP, FACC, FCCP is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American College of Physicians, American Heart Association, and American Thoracic Society
Disclosure: Actelion Grant/research funds Clinical Trials + honoraria; Ikaria Grant/research funds Clinical Trials + honoraria; Gilead Grant/research funds Clinical Trials + honoraria; Pfizer Grant/research funds Clinical Trials + honoraria; United Therapeutics Grant/research funds Clinical Trials + honoraria; Lilly Grant/research funds Clinical Trials + honoraria; Lung LLC Clinical Trials + honoraria; Bayer Grant/research funds Consulting; Medtronic Consulting fee Consulting; Novartis Consulting fee Consulting
Kurt Pflieger, MD, FAAP Active Staff, Department of Pediatrics, Lake Pointe Medical Center
Kurt Pflieger, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Emergency Physicians, American Heart Association, and Texas Medical Association
Disclosure: Nothing to disclose.
Gary Edward Sander, MD, PhD, FACC, FAHA, FACP, FASH Professor of Medicine, Director of CME Programs, Team Leader, Root Cause Analysis, Tulane University Heart and Vascular Institute; Director of In-Patient Cardiology, Tulane Service, University Hospital; Visiting Physician, Medical Center of Louisiana at New Orleans; Faculty, Pennington Biomedical Research Institute, Louisiana State University; Professor, Tulane University School of Medicine
Gary Edward Sander, MD, PhD, FACC, FAHA, FACP, FASH is a member of the following medical societies: Alpha Omega Alpha, American Chemical Society, American College of Cardiology, American College of Chest Physicians, American College of Physicians, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Society for Pharmacology and Experimental Therapeutics, American Society of Hypertension,American Thoracic Society, Heart Failure Society of America, Louisiana State Medical Society, National Lipid Association, and Southern Society for Clinical Investigation
Disclosure: Forest Labs Honoraria Speaking and teaching
Gary Setnik, MD Chair, Department of Emergency Medicine, Mount Auburn Hospital; Assistant Professor, Division of Emergency Medicine, Harvard Medical School
Gary Setnik, MD is a member of the following medical societies: American College of Emergency Physicians, National Association of EMS Physicians, and Society for Academic Emergency Medicine
Disclosure: SironaHealth Salary Management position; South Middlesex EMS Consortium Salary Management position; ProceduresConsult.com Royalty Other
Mark Spektor, DO Medical Director, Department of Emergency Medicine, Maimonides Medical Center
Mark Spektor, DO is a member of the following medical societies: American College of Emergency Physicians, American College of Physician Executives, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Medscape Salary Employment
Tetralogy of Fallot (TOF) in Adults
Research & References of Tetralogy of Fallot (TOF) in Adults|A&C Accounting And Tax Services
Source
0 Comments