Congestive Heart Failure with Tetralogy of Fallot Relieved by an Aortopulmonary Shunt

Congestive heart failure occurring in unoperated patients with otherwise uncomplicated tetralogy of Fallot (TF) is rare. We report the case of a boy who was first diagnosed as having TF when he presented at age 3 years with hypoxia and heart failure. Heart failure improved following an aortopulmonary shunt. We attributed his heart failure to cardiomyopathy associated with severe hypoxia. We believe that this report is the first one that attributes heart failure to hypoxia in an unoperated patient with tetralogy of Fallot.     

Exercise Capacity in Young Patients After Total Repair of Tetralogy of Fallot

Tetralogy of Fallot is the most common form of cyanotic congenital heart disease. Measurement of physical activity is usually performed as a routine part of the patient's cardiac evaluation. The aim of this study was to examine the exercise performance of young patients operated on for tetralogy of Fallot, assessing the possible influence of known negative prognostic factors related to the surgical repair. The study group comprised 41 consecutive patients (29 male and 12 female, ages 11.2 ± 3.9 years, range 6–16 years) operated on for tetralogy of Fallot. Patients in the study group were divided in subgroups in relation to the age of surgical intervention (before or after 2 years of life), the surgical approach (combined transatrial/transpulmonary approach or right ventriculotomy), and the presence of aortopulmonary shunts prior to performing total correction. Their data were compared with those of 33 aged-matched asymptomatic control subjects (19 male and 14 female, ages 11.9 ± 1.3 years, range 11–16 years). Blood pressure and heart rate measured at rest were similar between control and Fallot groups. A normal increase in systolic blood pressure was observed in response to exercise intensity for all subgroups. No significant difference between control and Fallot groups was found under conditions of mild or moderate exercise or for diastolic blood pressure at rest and in response to exercise. Lower maximal heart rate and systolic blood pressure values were recorded in all patients when compared with the control subjects. Significant differences in peak workload were detected between control and Fallot groups and between the control and each subgroup; however, no difference was found between subgroups. In conclusion, despite their very satisfactory clinical status, all patients showed a reduced peak workload, irrespective of the surgical approach, age at surgery, and aortopulmonary shunts prior to performing total correction.     

Clinical Applications of Cardiac Magnetic Resonance Imaging After Repair of Tetralogy of Fallot

In the past 15 years, cardiovascular magnetic resonance (MR) has evolved into an imaging technique that provides adequate, and in part unique, information on residual problems in the follow-up of patients operated for tetralogy of Fallot. Spin-echo or gradient-echo cine magnetic resonance imaging allow detailed assessment of intracardiac and large vessel anatomy, which is particularly helpful in Fallot patients with residual abnormalities of right ventricular outflow and/or pulmonary artery. Multisection gradient-echo cine MRI can be used to obtain accurate measurements of biventricular size, ejection fraction, and wall mass. This allows serial follow-up of biventricular function. MR velocity mapping is the only imaging technique available that provides practical quantification of pulmonary regurgitation volume. MR velocity mapping can also be used to quantify right ventricular diastolic function in the presence of pulmonary regurgitation.     

Course of Right and Left Ventricular Function in Patients with Pulmonary Insufficiency After Repair of Tetralogy of Fallot

Surgical repair of tetralogy of Fallot (TOF) frequently results in pulmonary valve insufficiency. Nevertheless, no serial information is available on the long-term impact of the valvular insufficiency on right and left ventricular function. Right and left ventricular ejection fraction was measured serially by radionuclide angiocardiography in 21 patients with at least moderate pulmonary insufficiency after repair of TOF. A baseline study was obtained an average of 1.2 years after repair, and a follow-up study was performed an average of 10.2 years after surgery. Changes in ventricular function over time and deviations from the normal range were analyzed. At baseline evaluation the mean right ventricular ejection fraction (RVEF; 0.52 ± 0.10) and left ventricular ejection fraction (LVEF; 0.68 ± 0.10) were normal. At the time of follow-up the mean RVEF had significantly decreased to 0.45 ± 0.09 (p < 0.01). The mean LVEF had decreased to 0.60 ± 0.11 (p < 0.02). This change was independent of the RVEF (r=−0.13). Eleven patients (52%) had an abnormal RVEF or LVEF at follow-up. Nineteen patients (90%) showed a decrease of 0.05 or more in RVEF, LVEF, or both between studies. These data suggest a negative impact of long-standing pulmonary insufficiency on right and left ventricular systolic function after repair of TOF. Therefore, continued surveillance of biventricular function in this patient population appears warranted.     

Electrophysiological Follow-Up of Tetralogy of Fallot

Arrhythmias and sudden death are well-recognized complications that occur late after correction of tetralogy of Fallot. This study, based on the literature and personal work, reviews the numerous investigations prompted by these complications and provides recommendations regarding follow-up. Conduction disturbances and ventricular arrhythmias are both common; although mostly asymptomatic, they are the likely cause of syncopy and sudden death. Supraventricular arrhythmias are less common but cause symptoms more frequently. Detecting patients at risk for life-threatening arrhythmias is an important task. The simple surface electrocardiogram (ECG) gives a host of information regarding patients at risk. Holter recordings, signal-averaged ECG, and invasive electrophysiological studies may all be helpful; the indications to perform these investigations are discussed and propositions are made for the follow-up of the postoperative patient. There is hope that a systematic approach along these lines, together with surgery done at an earlier age, will decrease the incidence of severe arrhythmias and sudden death in the future.     

Tetralogy of Fallot with Absent Pulmonary Valve and Bronchial Compression: Treatment with Endobronchial Stents

Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac lesion. The lesion includes ventricular septal defect, overriding aorta, and absence of the pulmonary valve, with resultant pulmonary incompetence. It has been suggested that the pulmonary incompetence induces intrauterine dilatation of the pulmonary artery, which leads to tracheobronchial compression. One of the presenting features in infants with APVS is severe airway obstruction, which may be difficult to manage. We report an infant who benefited from bilateral endobronchial endoscopic stent placement.     

Biventricular Long Axis Function After Repair of Tetralogy of Fallot

Right ventricular restrictive physiology is common after repair of tetralogy of Fallot and relates to exercise performance and symptomatic arrhythmias. In this study, we examined biventricular long axis function in an attempt to clarify further the mechanical substrate of this phenomenon. We studied prospectively 95 patients with tetralogy of Fallot (age range 1–44.3 years) at a median of 4.3 years after repair with Doppler and M-mode echocardiography. Pulmonary arterial, tricuspid, and mitral Doppler spectrals and 2-D guided M-mode recordings of ventricular minor and long axes were obtained with simultaneous phonocardiogram and respirometer recordings. Right ventricular restriction was defined by the presence of antegrade pulmonary arterial flow during atrial systole throughout the respiratory cycle. Restrictive right ventricular physiology was demonstrated in 36 (39%) [group 1] of the 92 patients in whom the data were analyzed. Left ventricular function (FS, isovolumic relaxation time and transmitral E wave deceleration time) was not different in the two groups (p < 0.1, p < 0.6, and p < 0.8, respectively). The presence of antegrade diastolic flow shortened the pulmonary regurgitation in the restrictive group (PR duration/√RR 10.7 ± 2.1 vs 12.1 ± 2.1, p < 0.01). There was delayed onset of shortening (97.4 ± 24 vs 88.8 ± 24 ms, p= 0.01), and the amplitude of right atrioventricular ring excursion, corrected for body surface area, was significantly lower during atrial systole in the restrictive group (0.43 ± 0.15 vs 0.54 ± 0.2 cm/m², p < 0.01). There was also a tendency toward a smaller ratio of right to left total atrioventricular ring excursion in the same group (1.14 ± 0.19 vs 1.22 ± 0.23, p= 0.1). Impaired long axis function in patients with restrictive right ventricular physiology following repair of tetralogy of Fallot is associated with abnormal diastolic filling and may contribute to the long-term cardioprotective effect of restrictive physiology by limiting the degree of right ventricular dilatation.     

Persistent Left Fifth Aortic Arch Associated with Tetralogy of Fallot

A case of persistent left fifth aortic arch, forming a congenital ``double-lumen' aortic arch, has been diagnosed on angiocardiography during life. It appeared as an unusual vascular structure running inferiorly and parallel to the ``real' aortic arch from the innominate artery to the left subclavian artery superior to the pulmonary artery. This anomaly was found in the setting of tetralogy of Fallot, an association never described before, with patent ductus arteriosus (previously reported in most cases). The left aortic arch in this case was not a source of pulmonary circulation, as described in previous cases with pulmonary atresia and ventricular septal defect, but was a systemic-to-systemic connection without functional relevance.     

Tetralogy of Fallot with Levoatrial Cardinal Vein

A levoatrial cardinal vein is a rare congenital anomaly of the systemic veins. It is frequently associated with left-side obstructive anomalies. We report a case of tetralogy of Fallot with a levoatrial cardinal vein. The innominate vein was absent and two left upper pulmonary veins connected to this vein. There were no left-side obstructive anomalies.     

Diagnostic and Interventional Trends in Tetralogy of Fallot and Transposition of the Great Arteries in a Population-Based Study

The goal of this study was to analyze diagnostic and interventional trends in tetralogy of Fallot and transposition of the great arteries in Malta for individuals born during 1920–1994 and calculate birth prevalences. The design was population based, in the setting of a regional hospital providing exclusive diagnostic and follow-up services for the entire population of Malta. Data collection and analysis was retrospective and included comparison with earlier epidemiological studies. Patients included were all Maltese live births diagnosed as having tetralogy of Fallot (TF) and transposition of the great arteries (TGA) born by the end of 1994. There were 109 cases of TF and 30 cases of TGA (n= 30). A significant decline in age at diagnosis and age at surgery was found for both lesions (p < 0.0001), which was associated with a significant decline in perioperative mortality (p= 0.005). Underascertainment was present prior to 1980, however, more cases were diagnosed due to improvement of postmortem services in the early 1980s followed by improvement of clinical services with eventually exclusive premortem diagnosis. The birth prevalence of TGA was 0.31/1000 live births, well within the range described in previous studies, unlike tetralogy of Fallot, which has been found in excess in this population in an earlier study. TF and TGA, the two lesions which comprise the majority of cyanotic congenital heart disease presenting in infancy, have been diagnosed and have undergone intervention at progressively earlier ages over the period under study. This decline was associated with a declining perioperative mortality.     

Enterococcus avium Endocarditis in an Infant with Tetralogy of Fallot

We report a case of infective endocarditis secondary to Enterococcus avium in a 1-year-old infant with tetralogy of Fallot and a Blalock–Taussig shunt. To our knowledge, this is the first case of E. avium endocarditis to be reported.     

Congenital Pulmonary Steal Phenomenon Associated with Tetralogy of Fallot, Right Aortic Arch, and Isolation of the Left Subclavian Artery

Isolation of the left subclavian artery has been reported in cases of tetralogy of Fallot with a right aortic arch. As the isolated left subclavian artery is supplied by the left vertebral artery (in which the blood flow direction is inverted), this anomaly is usually responsible for a congenital subclavian steal phenomenon. In our case the isolated left subclavian artery was connected by a patent ductus arteriosus to the main pulmonary artery. Therefore the left vertebral artery did not supply the distal left subclavian artery but supplied the main pulmonary artery (congenital pulmonary artery steal).     

Double-Outlet Right Ventricle (Tetralogy of Fallot Type) Associated with Anomalous Origin of the Left Coronary Artery from the Right Pulmonary Artery: Report of Successful Total Repair in a 2-Month-Old Infant

The case reported herein demonstrates the rare association of double-outlet right ventricle (tetralogy of Fallot type) with anomalous origin of the left coronary artery from the pulmonary artery. It is the first reported successful total surgical repair in an infant.     

Determinants of Aerobic Capacity During Exercise Following Complete Repair of Tetralogy of Fallot with a Transannular Patch

Exercise capacity and the causes of its limitation following repair of tetralogy of Fallot have been studied in heterogeneous populations. Study populations have been grouped together regardless of the type of repair and residual hemodynamic abnormalities. To better understand the factors limiting aerobic exercise capacity in patients repaired with a transannular patch, 37 patients with a transannular patch and no residual pulmonary stenosis underwent resting spirometry and treadmill exercise testing. Maximal oxygen consumption and oxygen consumption at anaerobic threshold were measured in all patients to assess aerobic capacity. Patients were subdivided by gender. Resting spirometry measurements tended to be lower in both genders compared to healthy controls but did not correlate with any measurement of aerobic capacity. Maximal oxygen consumption and anaerobic threshold were significantly less in the female than the male population. A quadratic relation between maximal oxygen consumption and age at exercise testing existed for both genders but peaked at an earlier age and was significantly less in the female population. There was a significant negative correlation between maximal oxygen consumption and echocardiographically estimated right ventricular inflow volume index in the female population only. These data suggest that in patients with tetralogy of Fallot repaired with a transannular patch aerobic capacity is limited primarily by cardiac function, but that gender differences are due to noncardiac causes.     

Histopathologic Changes in the Infundibular Septum After Balloon Pulmonary Valvuloplasty in Tetralogy of Fallot

Balloon pulmonary valvuloplasty (BPV) has been applied to tetralogy of Fallot (TOF) as a palliative procedure. To investigate the histopathologic changes by BPV in the infundibular septum of TOF, we performed histopathologic examinations of the infundibular septum resected at corrective surgery. The subjects were 5 patients with TOF, who underwent BPV at the median age of 2.2 months and the corrective surgery at the median age of 15.0 months (BPV group), and 4 patients with TOF who had no prior BPV and who underwent the corrective surgery at the median age of 14.5 months (control group). There was no significant difference between the two groups in the endocardial thickness, myocardial vacuole degeneration, or fraction of fibrous and interstitial space. However, the specimens from 3 patients in the BPV group had localized dense fibrous lesions in the myocardium. The findings in this limited sample suggest that BPV for TOF does not produce overall histopathologic alterations such as fibrosis, thick endocardium, and myocardial vacuole degeneration, but may sometimes damage the myocardium of the infundibular septum resulting in the formation of localized dense fibrous lesions. The clinical significance of this damage is still unknown, and further cases should be investigated.     

Adverse Hemodynamic Effects Observed with Inhaled Nitric Oxide After Surgical Repair of Total Anomalous Pulmonary Venous Return

The following is a case report of a 1-month-old patient who developed adverse hemodynamic sequelae during the use of nitric oxide (NO) in the postoperative period for pulmonary hypertension after correction of total anomalous pulmonary venous return. At the time of diagnosis, the patient had evidence of systemic right ventricular pressures estimated by continuous-wave Doppler. He was sedated and paralyzed for hyperventilation in preparation for surgery and underwent pulmonary vein confluence to left atrial anastomosis. Postoperative pulmonary hypertension was managed by hyperventilation, sedation, and paralysis until a sudden onset of systemic-level pulmonary pressure required NO therapy. Satisfactory results were obtained in minutes, but a rebound pulmonary hypertension occurred with concomitant systemic hypertension and no radiographic changes. We suspected left atrial hypertension secondary to a sudden increase in pulmonary blood flow to an noncompliant left ventricle. Discontinuation of NO resulted in stabilization of the hemodynamic profile of the patient and he continued to be managed with paralysis, hyperventilation, and sedation. Based on this experience we suggest that NO should be used with caution in patients with obstructive lesions at the atrial level prior to surgery (mitral valve stenosis and cor triatriatum) or in patients with a poorly compliant left ventricle (cardiomyopathy and left ventricular dysfunction). These entities are unable to tolerate a sudden increase in pulmonary blood return thus creating paradoxical pulmonary hypertension.     

Pulmonary Atresia with Intact Ventricular Septum and Major Aortopulmonary Collaterals: Association with Deletion 22q11.2

We describe the first association of pulmonary atresia, intact ventricular septum, and absent central pulmonary arteries with deletion 22q11.2. The pulmonary blood flow was derived from major aortopulmonary collaterals. The role of the deletion in pulmonary arborization is discussed.     

Anomalous Origin of the Left Coronary Artery in Tetralogy of Fallot Associated with Abnormal Mitral Valve Pathology

The association of anomalous left coronary artery with congenital heart disease is a rare occurrence. Seven cases of anomalous left coronary artery associated with tetralogy of Fallot have been reported in the literature. We report a unique case with severe mitral valve abnormality that precluded standard surgical repair.     

Reprint of Staged Repair of Tetralogy of Fallot with Pulmonary Atresia and Major Aortopulmonary Collateral Arteries

Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs) is a rare but important anatomical configuration. Unifocalization is one surgical procedure that creates an adequate pulmonary circulation in the setting of MAPCAs. In this text, the variable anatomy of MAPCAs and the correspondingly variable unifocalization procedures are described in seven case presentations.