Samuel Menahem

Early surgical closure of a large ventricular septal defect: Influence on long-term growth

The pre- and postoperative growth patterns of 52 otherwise normal infants undergoing primary surgical closure of a large ventricular septal defect before 7 months of age were reviewed. Serial measurements of weight, length and head circumference were compiled for all patients preoperatively and in 46 long-term survivors and were expressed as Z scores (in standard deviations from the mean for age and gender). By the time of surgery at a mean age of 0.33 year, the mean weight, length and head circumference Z scores of all 52 infants were -2.9, -0.9 and -0.6, respectively, and were all significantly below normal (p less than 0.001). At a mean age of 5.7 years, the mean weight, length and head circumference Z scores of 35 patients of normal birth weight were normal or varied only marginally from those of the reference population (-0.4, -0.1 and +0.5, respectively; p less than 0.02, p greater than 0.05 and p = 0.008, respectively) and did not differ significantly in any variable from those of 44 normal siblings. However, among 11 infants with a low birth weight, all three variables remained abnormal at long-term follow-up when compared with the reference population (-1.7, -1.7 and -0.9, respectively; p less than 0.001 for each) and 22 normal siblings (p less than or equal to 0.008).(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac Function and Arterial Biophysical Properties in Small for Gestational Age Infants: Postnatal Manifestations of Fetal Programming

OBJECTIVE To investigate the differences in cardiac function and arterial biophysical properties between term-born appropriate for gestational age (AGA) infants and small for gestational age (SGA) infants. Our hypothesis was that adaptation to intrauterine growth restriction induces changes in cardiac and arterial indices. STUDY DESIGN This was a prospective observational echocardiographic evaluation of cardiac and arterial indices in SGA infants and AGA infants. Demographic and echocardiographic data were compared between 20 inborn term SGA infants with birth weight <3rd percentile for gestational age and 20 AGA infants. RESULTS The Ponderal index was significantly lower and blood pressure was significantly higher in the SGA infants compared with the AGA infants. Left ventricular output was lower in the SGA infants (170 ± 31 mL/kg/min vs 197 ± 39 mL/kg/min). Diastolic dysfunction was greater in the SGA infants (ie, reduced E and A wave velocities, higher E/A ratio [1.08 ± 0.16 vs 0.85 ± 0.07], and prolonged isovolumic relaxation time [73 ± 6.2 ms vs 62.6 ± 3.6 ms]). Aortic intima-media thickness was significantly greater in the SGA infants (822 ± 105 μm vs 694 ± 52 μm), as were arterial wall stiffness index and input impedance. CONCLUSION Cardiac function and arterial biophysical properties were altered in the SGA infants. The findings complement the information on the association between in utero growth and cardiovascular morbidity in later life.

Clinical Features and Management of Isolated Cleft Mitral Valve in Childhood

OBJECTIVES We reviewed an institutional experience of isolated cleft mitral valve (ICMV), its clinical features, and management in a pediatric population. BACKGROUND As ICMV is relatively uncommon, earlier reports highlighted its anatomical and echocardiographic features. Few studies have collated their clinical features with their outcome. METHODS All patients with ICMV were retrospectively reviewed. Patients who were considered to have an atrioventricular septal defect or variant were excluded. RESULTS Twenty patients (9 male, 11 female) were diagnosed with ICMV. Seven patients had associated cardiac lesions. The median age of diagnosis was 5.2 years (range 0.4 to 13.6 years). Echocardiography aided by color Doppler demonstrated the ICMV in all patients. However, an incomplete diagnosis was made in 4 of 20 patients before surgery. The severity of the mitral regurgitation (MR) at presentation was mild in 11, moderate in 8, and severe in 1 patient. In the 13 patients without associated cardiac lesions, 5 underwent mitral valve (MV) repair at median age of 5.2 years (range 1.2 to 7.7 years) for moderate to severe MR, 4 being symptomatic. The severity of the MR in seven of the eight unoperated patients has remained unchanged over the follow-up period (median 8.3 years, range 0.7 to 14.4 years). In total, 10 patients underwent MV repair (median 6.4, range 0.4 to 13.8 years). No patient required MV replacement. None of the 10 patients had more than mild MR over the follow-up period (median 0.6, range 0.2 to 11.0 years). CONCLUSIONS Now readily diagnosable by echocardiography, ICMV is a correctable cause of MR with a good outcome. Surgery is indicated in those patients with moderate to severe MR and probably should be done early following diagnosis.

Surgical intervention for infective endocarditis in infancy and childhood.

BACKGROUND Infective endocarditis is an uncommon but serious disease in children. Optimal treatment strategy, especially surgical indications, continues to evolve. METHODS Retrospective review of 98 patients treated for infective endocarditis during the past 13 years at the Royal Childrens Hospital, including medically and surgically treated patients. RESULTS Thirty of 98 patients had surgical intervention with 6.7% hospital mortality, and 76% survival probability at 45 months. The remaining patients were treated medically, with 10% hospital mortality and 52% 5-year survival probability. The incidence of structural heart disease, congestive heart failure, and spectrum of organisms was similar in the two groups. CONCLUSIONS Despite advances in antibiotic therapy, early surgical intervention is required in a significant subset. Concurrent intracardiac repair may be appropriate.

Infant Colic, Distress, and Crying

The literature regarding infant colic is critically reviewed. Although there have been a number of theories proposed as to etiology of colic, the literature is characterized by difficulties in definition, methodologic problems, and numerous claims as to both etiology and management that are anecdotal. Infant colic is best conceptualized as the end result of a complex transaction between the infant and his environment, with multiple factors responsible for the crying and distress of an infant. The most important factors in appropriate intervention are a physicians receptivity and sensitivity toward the stressed mother, together with an interested and practical approach to providing adequate support while delineating the individual stresses acting on both mother and baby. Future research is needed to delineate markers for those subgroups of infants who may present with crying as a manifestation of specific clinical situations.

Interrupted aortic arch in infancy: A 10-year experience

SummaryFifty infants with interrupted aortic arch (IAA), admitted between 1979 and 1988, were reviewed. They usually presented early in severe cardiac failure or shock. In the initial 5-year period, 17 of the 21 infants underwent diagnostic or confirmatory cardiac catheterization, in contrast with the latter 5 years when only eight of the subsequent 29 patients underwent catheterization. Since 1987, all patients underwent surgery after cross-sectional echocardiography. Fifteen infants had a type A IAA and 35 had type B. All had associated cardiac anomalies. Four infants were not operated on.In the initial 5-year period, of 17 infants who were surgically treated, four had a one-stage total repair, the remaining had a two-stage repair with initial reconstruction of the arch and pulmonary artery banding. There was an overall surgical mortality of 65%, reffecting the precarious state of many of these infants before surgery with a significant contribution from unrelieved subaortic stenosis.In the latter 5-year period, 29 underwent surgery, 22 had a one-stage total repair. There were three deaths, all in infants whose active treatment was withdrawn. The outcome of the survivors has generally been good, subsequent surgery being mainly related to the associated anomalies (e.g., recurrent subaortic stenosis, conduit replacement).Over this 10 year period the greater accuracy of noninvasive diagnoses, and perioperative intensive care, have led to an improvement in the preoperative state of these infants. Singlestage total repair is our procedure of choice.

Infective endocarditis on an occluder closing an atrial septal defect.

Closure of atrial septal defects be means of intravenous catheterisation has been undertaken using a variety of devices as an alternative to surgical closure. We describe the first case, to the best of our knowledge, of infective endocarditis complicating a successful transcatheter closure. This highlights the potential risk of this procedure, and emphasises the need for appropriate antibiotic prophylaxis until complete endothelialization of the device has occurred.

Long-term follow-up after two coronary repair of anomalous left coronary artery from the pulmonary artery.

Abstract A retrospective analysis of ten patients with anomalous left coronary artery arising from the pulmonary artery operated between 1979 and 1990 was undertaken. All presented with evidence of left ventricular dysfunction and “ischemic” mitral regurgitation. Surgical repair consisted of an aortopulmonary tunnel (Takeuchi) procedure in eight and direct left coronary artery reimplantation in two. Two patients required postoperative support with a left ventricular assist device. There were no operative or late deaths (CL 0% to 17%) for a follow‐up of over 670 patient months. All patients are in New York Heart Association Class I or II, though two patients are still receiving anticongestive medications. One patient has required further surgery for pulmonary artery stenosis, and another has had a mitral valve replacement because of severe mitral regurgitation. One additional patient has moderate‐to‐severe residual mitral regurgitation and two have a trivial left coronary to main pulmonary artery fistula. All have a patent, nonstenotic left coronary artery and much improved left ventricular function and perfusion as assessed by echocardiography, thallium scan, gated blood pool scan, and angiography. There have been no documented arrhythmias, clinically or on Hotter monitoring. The ECGs have shown resolution or improvement of the initial changes of ischemia/infarction in all patients. Chest X‐rays have shown normalization of cardiothoracic ratio in eight of ten patients. Excellent early and late results can be achieved following timely surgical repair. Marked improvement in left ventricular function has been observed in patients with poor preoperative left ventricular function, even in the presence of extensive ischemia/infarction.

The Prevalence and Predictors of Anxiety and Depression in Adolescents with Heart Disease

OBJECTIVE To evaluate the prevalence and contributory factors of depression and anxiety in adolescents with heart disease. STUDY DESIGN A cohort of adolescents was recruited from an ambulatory cardiology clinic at a tertiary center and from such clinics run privately. Questionnaires included the Hospital Anxiety and Depression Scale, Multidimensional Scale of Perceived Social Support, Life Orientation Test-Revised, and Sense of Coherence-13. RESULTS A total of 119 adolescents aged 12-20 years were recruited. Most of the participants (90%) had congenital heart disease. Nearly all (98%) were in New York Heart Association Functional Classification class I. Approximately one-half of the participants had had previous surgeries and/or catheter interventions. Eleven patients (9%) were above the cut-off score for likely depression, and 32 (27%) had likely anxiety. A feeling of optimism was the most significant factor in reducing anxiety and depression, followed by good social support and a strong sense of coherence (multiple regressions, β = -0.38, -0.2, -0.17, respectively). CONCLUSIONS Depression and anxiety were found to be common in a relatively well adolescent population with heart disease. None of those affected had been clinically assessed and appropriately treated by a mental health professional. Protective factors against depression and anxiety need be developed as well as appropriate assessment and intervention.

Coronary arterial fistulas in childhood.

We reviewed 16 patients with coronary arterial fistulas seen between 1976 and 1997, and aged 2 days to 16 years, with a median age of 3.2 years. Only four patients were symptomatic: two had heart failure, one had exertional dyspnoea, and one infective endocarditis. The fistulas originated from the right coronary artery in seven patients, from the left coronary artery in seven, from both coronary arteries in one patient, while the origin was not clearly defined in the final patient. Associated cardiac anomalies were discovered in six patients, with three of the fistulas being diagnosed at the same presentation. Cross-sectional echocardiography had revealed a dilated coronary artery in 7 out of 11 subjects. The ratio of pulmonary to systemic flows ranged between 0.9 to 3.0, with a median of 1.5. Ten patients were referred for corrective surgery without any mortality. Trans-catheter closure was successfully undertaken in one patient, while spontaneous closure of the fistula was noted in two patients. We conclude that coronary arterial fistulas, although rare and potentially serious, are generally treatable.