Victor D. Menashe

A population-based prospective evaluation of risk of sudden cardiac death after operation for common congenital heart defects

OBJECTIVES This study sought to define 1) the risk of sudden death after operation for common congenital heart defects; and 2) factors associated with an increased risk of sudden death. BACKGROUND Although the prognosis for patients with congenital heart defects is improved by surgical treatment, they remain at a well recognized but poorly defined risk of late sudden death. METHODS This population-based study evaluated all patients < 19 years old undergoing surgical treatment of common forms of congenital heart disease in the state of Oregon between 1958 and 1996. Patients were identified retrospectively through 1958, with prospective biannual follow-up beginning in 1982. The incidence and cause of late sudden death were evaluated for 3,589 patients surviving operation for the following defects: atrial, ventricular and atrioventricular septal defects; patent ductus arteriosus; pulmonary stenosis; aortic stenosis; coarctation of the aorta; tetralogy of Fallot; and D-transposition of the great arteries. RESULTS There were 41 unexpected late sudden deaths during 45,857 patient-years of follow-up, an overall event rate of 1/1,118 patient-years. Thirty-seven of the 41 late sudden deaths occurred in patients with aortic stenosis, coarctation, transposition of the great arteries or tetralogy of Fallot, an event rate of 1/454 patients-years. In contrast, only four sudden deaths occurred among the other defects, an event rate of 1/7,154 patient-years (p < 0.01). The risk of late sudden death increased incrementally 20 years after operation for tetralogy of Fallot, aortic stenosis and coarctation. However, risk was not dependent on patient age at operation or surgical era. The causes of sudden death were arrhythmia in 30 patients, circulatory (embolic or aneurysm rupture) in 7 and acute heart failure in 4. CONCLUSIONS The risk of late sudden death for patients surviving operation for common congenital heart defects is 25 to 100 times greater than an age-matched control population. This increased risk is primarily represented by patients with cyanotic or left heart obstructive lesions. The risk of sudden death appears to be time dependent, increasing primarily after the second postoperative decade.

Hypoventilation and cor pulmonale due to chronic upper airway obstruction

A syndrome of chronic upper airway obstruction, hypoventilation, and cor pulmonale seen in 2 patients is described. These children both manifested varying degrees of CO 2 retention, pulmonary hypertension, and right ventricular hypertrophy. Relief of the upper airway obstruction by tonsillectomy and adenoidectomy resulted in regression of the presenting symptoms and signs.

Aortic root dilatation and mitral valve prolapse in Marfan's syndrome: an ECHOCARDIOgraphic study.

Echocardiographic and phonocardiographic findings in 35 patients with Marfans Syndrome and ten patients without Marfans or other clinically apparent connective tissue disorders but with angiographic and echocardiographic evidence of mitral prolapse are reported and compared. Echocardiography revealed aortic root dilatation and/or mitral valve prolapse in 97% of the patients with Marfans Syndrome. Aortic root dilatation was found in 60% of this group (74% of males, 33% of females) while mitral valve prolapse was found in 91% (87% of males, 100% of females). The incidence of aortic dilatation and mitral prolapse in patients with Marfans Syndrome was essentially equal in children and adults of the same sex. None of the nine adults or one child with mitral prolapse but without evidence of Marfans Syndrome or other clinically apparent connective tissue disorder had aortic root enlargement. Auscultatory examination and phonocardiography revealed abnormalities in 54% of the patients with Marfans Syndrome. Aortic regurgitation was found in 23% of this group (35% of males, 0% of females) while mitral regurgitation and/or mitral clicks were found in 46% (39% of males, 58% of females). Aortic regurgitation was much more frequent in adult males with Marfans Syndrome (7/14, 50%) than male children (1/9, 11%), while the incidence of abnormal mitral sounds was essentially the same in adults (33% of males, 60% of females) and children (43% of males, 57% of females) of the same sex with Marfans Syndrome. Abnormal mitral sounds were more frequent in patients without Marfans who had mitral prolapse (90%) than in those with Marfans (46%). It appears that cardiac abnormalities are a consistent manifestation of Marfans Syndrome and that ultrasound is a more sensitive indicator of these abnormalities in such patients than auscultation or phonocardiography.

Cardiac Surgery for Grown-Up Congenital Heart Patients: Survey of 307 Consecutive Operations from 1991 to 1994

The cardiac surgery performed from 1991 to 1994 in a unit dedicated specifically for grown-up congenital heart (GUCH) patients was reviewed to determine the frequency of various procedures, incidence of first and reoperations, early mortality, and its determinants. The 295 patients, aged 16 to 77 years (31 +/- 13), had 307 operations. First operations (n = 128, 42%) were most commonly for closure of atrial septal defect (n = 40), aortic valve replacement (n = 31) or repair of aortic coarctation (n = 14). Reoperations were more frequent (n = 179, 58%) and divided among first corrective repair (n = 49), reoperation after corrective repair (n = 115), and further palliation (n = 15). First corrective surgery was mainly for aortic valve disease (n = 17), Fallot (n = 7), and lesions needing a Fontan procedure (n = 5). Reoperations after corrective repair were needed for aortic valve disease (n = 43), right-sided conduit (n = 30), or recoarctation (n = 11). Early mortality was influenced by presence of central cyanosis (9 of 49, 18% in cyanotic patients; 12 of 258, 5% in acyanotic; p <0.001), increased number of previous operations (0 = 4%, 1 = 7%, 2 = 11%, >2 = 13%; p = 0.003), and increasing age of patients. Cyanotic patients had more serious postoperative complications: pleural and pericardial effusions, severe bleeding, renal insufficiency, and sepsis, and their hospital stay was longer compared with acyanotic patients (20 +/- 17 vs 11 +/- 8 days; p <0.001). In GUCH patients, reoperations cause the largest demand on cardiac surgical services. Increased survival of patients with complex cardiovascular malformations brings difficult challenges not only to cardiologists but also to cardiovascular surgeons. There is a need to provide continued highly specialized care. Resources, patients, and funding should be concentrated in a few designated centers.

Late complications after femoral artery catheterization in children less than five years of age

Fifty-eight children who underwent diagnostic femoral artery catheterization before 5 years of age, from 5 to 14 years before the study, were randomly selected from approximately 300 surviving patients undergoing diagnostic femoral artery catheterization at our institution during the interval. Each patient underwent vascular laboratory segmental pressure and waveform examination and arterial duplex scanning, as well as lower extremity bone length radiographs, which were considered positive if the catheterized leg was greater than or equal to 1.5 cm shorter than the opposite leg. Thirteen children who had only venous catheterization served as controls. No arterial abnormalities were present in the control patients (mean ankle/brachial index, 1.01). Arterial occlusion was present in both limbs of five patients who had bilateral diagnostic femoral artery catheterization and in 14 limbs of 51 patients who had unilateral diagnostic femoral artery catheterization. Thus arterial occlusion was present in 33% of patients (19 of 58) and in 37% of limbs (24 of 65). The mean ankle/brachial index in the catheterized limbs was 0.79. Leg growth retardation was present in four limbs (8%) of 51 children undergoing unilateral diagnostic femoral artery catheterization and in one (8%) control patient. The inverse relationship between ankle/brachial index and leg growth retardation was significant (R = 0.47, p less than 0.0005). Only one patient had symptoms of arterial occlusion (claudication), and one patient had symptoms of leg growth retardation (gait disturbance). We conclude that arterial occlusion is common after diagnostic femoral artery catheterization in children less than 5 years of age, but that excellent collateral supply prevents leg growth retardation and/or symptomatic arterial insufficiency in most children.(ABSTRACT TRUNCATED AT 250 WORDS)

Ehlers-Danlos syndrome associated with multiple pulmonary artery stenoses and tortuous systemic arteries

A young girl with Ehlers-Danlos syndrome had evidence of multiple severe peripheral pulmonary artery stenoses and of tortuosity of the aorta and its major thoracic divisions, including the coronary arteries. It appears likely that the basic defect of Ehlers-Danlos syndrome may involve many arteries and may cause important vasular obstruction.

Total anomalous pulmonary venous connection in neonates and young infants: Repair in the current era☆

Total anomalous pulmonary venous connection has been one of the more challenging congenital heart defects in newborns and young infants despite improvements in surgical technique, cardiac anesthesia, neonatal myocardial preservation, and postoperative care. Since 1981, 30 patients with total anomalous pulmonary venous connection have undergone primary total correction. Mean age at operation was 28 +/- 6 days and mean weight, 3.3 +/- 0.7 kg. Essential features of the surgical approach in these small patients included early surgical intervention, profound hypothermia with total circulatory arrest, cardioplegic myocardial preservation, and a wide posterior anastomosis. Operative mortality was 13% +/- 6%. All four deaths were in patients having emergency operation within 24 hours of the surgical consult and requiring ventilator support preoperatively. The mean follow-up is 47 +/- 7 months. There have been two late deaths, and the 7-year survival rate is 79% +/- 8%. There have been two reoperations, and 91% +/- 6% of the patients are reoperation free at 7 years. Only 1 of the 24 surviving patients is symptomatic. Growth in survivors is closely monitored. The height growth percentile is less than 5% in 15% +/- 8% of survivors and the weight growth percentile, less than 5% in 17 +/- 8%. During the past decade, with a consistent surgical approach to neonates and infants with total anomalous pulmonary venous connection, it has been possible to achieve low early mortality, low attrition, and excellent late results.

Serial cardiac catheterizations and exercise hemodynamics after correction of tetralogy of Fallot: average follow-up 13 months and 7 years after operation.

Eleven patients had right heart catheterization an average of 13 mo after total correction of tetralogy of Fallot, and the procedures were then repeated an average of 7 years postoperatively. In the intervening time there was generally no important change in the pressure gradient between the right ventricle and pulmonary artery or in right ventricular systolic pressure. Mean right atrial pressure tended to fall with time. Arteriovenous oxygen difference at rest was lower at the second study, and the resting cardiac output was generally normal. One patient with a persistent ventricular septal defect had progressive hemodynamic deterioration between the two studies. Exercise performance up to 10 years postoperatively was also assessed. The relationship between oxygen consumption and cardiac output was usually normal, but exercise magnified the right hearts filling pressure abnormalities. In the absence of an easily demonstrable ventricular septal defect, right heart hemodynamics were either stable or improved up to 10 years postoperatively. The exercise response of cardiac output was usually normal at moderate work loads.

Total correction of tetralogy of Fallot in infancy. Postoperative hemodynamic evaluation.

Total intracardiac repair of symptomatic tetralogy of Fallot was accomplished in twenty-nine infants under two years of age with a mortality of six per cent. All twenty-seven survivors are asymptomatic. Postoperative hemodynamic evaluation of 17 randomly selected children was performed at least 12 months following surgery. Pulmonary regurgitation was present in 12 of the 17 children but was well tolerated, with only minimal cardiac enlargement and in no case were there symptoms or important hemodynamic consequences. Growth of the pulmonary artery with respect to the aorta occurred. Normal left ventricular performance was indicated by normal ejection fraction and normal systemic arterial pressure. Intellectual and social development were indistinguishable from a “normal” group of randomly selected children as assessed by detailed psychological testing.

Total correction of tetralogy of Fallot: Complications and results

Abstract An analysis of the postoperative course in twenty-eight patients who had complete correction of tetralogy of Fallot is presented. Pertinent preoperative findings are summarized and the operative procedure is briefly described. During or following surgery, seven patients had a syndrome of low cardiac output, which was lethal in four. Factors which may have contributed to the development of this state are discussed. The operative mortality rate was 14 per cent. One patient died during re-operation for persistence of the ventricular septal defect; the over-all mortality rate was 18 per cent. Congestive heart failure developed in 42 per cent of surviving patients. This was transient in some and had a more protracted course in others. All patients recovered and late improvement was impressive. Possible mechanisms for this incidence of heart failure are presented. The ultimate result from surgery was considered excellent in the majority of cases. It is concluded that total correction is feasible in most patients with tetralogy of Fallot, with an acceptable surgical mortality.