Thomas C. Martin

Dilated cardiomyopathy in infants and children.

The outcome of medical treatment of dilated cardiomyopathy in infants and children was reviewed to develop a predictive index for selection of patients likely to benefit from cardiac transplantation. The clinical findings, laboratory investigations, treatment and outcome of 20 patients (Group 1) less than 2 years of age at presentation and 12 patients (Group 2) greater than 2 years of age at onset were compared. Of 20 Group 1 patients, 5 (25%) died. Available autopsies (four patients) showed endocardial fibroelastosis. Of 15 survivors, 10 showed improvement in cardiac status and 5 remained unchanged. Ninety-three percent of survivors had dilated cardiomyopathy consistent with endocardial fibroelastosis by angiocardiography. All 12 Group 2 patients died. In addition to age at presentation and poor outcome, Group 2 differed from Group 1 in having a higher incidence of other family members with cardiomyopathy, more significant rhythm disturbances at presentation and a more rapid course to death. Risk factors of poor outcome in both groups included persistent cardiomegaly and the development of significant arrhythmias by Holter electrocardiographic monitoring. Cardiac transplantation is recommended for children with dilated cardiomyopathy presenting after age 2 years who survive 1 month. Those patients less than 2 years old at presentation whose condition has not improved after 1 year and who have persistent cardiomegaly or complex ventricular arrhythmias may also benefit from transplantation.

Spontaneous closure of secundum atrial septal defect in infants and young children

The records of 264 pediatric patients with uncomplicated ostium secundum atrial septal defect (ASD) were reviewed. Eighty-seven patients were younger than age 4 years at the time of cardiac catheterization. Subnormal weight gain, frequent pneumonia, cyanosis or tachypnea were present in 26 patients (30%). Of the 36 infants at catheterization, 17 (48%) had the previously described symptoms, including 12 (33%) who had congestive heart failure. Eight of the 36 infants were found to have closed their defect at a subsequent catheterization. Six of 18 patients who underwent cardiac catheterization between 1 and 2 years of age also had spontaneously closed their ASD at subsequent study. Statistical analysis of hemodynamic data revealed no difference (except a smaller shunt size) between ASDs that closed and those that did not in patients who were less than 4 years at initial catheterization. Analysis of hemodynamic data revealed no statistical differences between groups of patients with an ASD who were younger than and those older than 4 years at time of diagnostic study. Patients with ASDs that closed were significantly different from patients with atrial level shunting thought to be secondary to a valve-incompetent foramen ovale with respect to age at initial study (11 versus 2 months, p less than 0.001), mean left atrial pressure (7.7 versus 12.3 mm Hg, p less than 0.02) and difference between mean right and left atrial pressures (1.0 versus 4.2 mm Hg, p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Evaluation of pulmonary arterial morphology in cyanotic congenital heart disease by magnetic resonance imaging

Before and after surgical therapy, the anatomy of the pulmonary arteries in cyanotic congenital heart disease is often distorted. Pulmonary arterial anatomy was evaluated by magnetic resonance imaging (MRI) and angiography in 20 patients, ages 3 months to 20 years, with cyanotic heart disease associated with decreased pulmonary blood flow. Excellent correlation between MRI and angiographic estimates of pulmonary artery diameter was obtained (main pulmonary artery, r = 0.96; right pulmonary artery, r = 0.93; left pulmonary artery, r = 0.96). A similar excellent correlation (kappa = 0.83) was found in the assessment of the presence and severity of proximal pulmonary arterial stenoses. However, stenoses in the peripheral pulmonary arteries visualized with angiography were missed with MRI. MRI and angiography showed complete agreement in determining the patency of 11 surgical shunts. MRI did not demonstrate all of the systemic collateral vessels present with angiography, and the distal connections of collaterals were not detected with MRI. MRI is comparable to angiography in the evaluation of central pulmonary arterial anatomy over a wide range of ages. These findings suggest an important role for noninvasive MRI in the serial evaluation of pulmonary artery morphology in patients with cyanotic congenital heart disease before and after surgical repair.

Management and follow-up of arterial thrombosis in the neonatal period

The management and follow-up of 12 patients with major aortic thrombus formation occurring in the neonatal period between 1982 and 1987 are reported. Umbilical arterial catheters were inserted in 8 of the 12 patients before thrombus formation. Two patients had congenital thrombi. Hypertension, oliguria, hematuria, and elevated blood creatinine concentration were found at the time of diagnosis of the thrombus; nine of the patients had a patent ductus arteriosus. Supportive care was instituted in seven patients who were hemodynamically stable. Five of the patients had congestive heart failure, shock, or both, and were treated with surgical thrombectomy. Thrombolytic therapy was not used in either group. The five surgically treated patients and six of seven medically treated patients survived. Ultrasound examination suggested resolution of the thrombus in all survivors in 6 to 30 days. Sequelae from thrombus formation were present in all patients at the time of discharge and included hypertension in 9 of the 11 survivors and decreased renal function in six of them. Follow-up at 1 to 3 years revealed normal blood pressure, good growth, and good renal function in 10 of the survivors.

Primary surgical closure of large ventricular septal defects in small infants

Herein, a policy of primary surgical closure of large ventricular septal defects in infants is reviewed. Forty-eight infants met criteria for inclusion in the study, and were divided into two groups based on weight: group 1 infants weighted 4 kg or less (n = 23), and group 2 infants weighed more than 4 kg (n = 25). Both groups had similar variation in ventricular septal defect location (paramembranous versus muscular) and number (single versus multiple), as well as incidence of major associated extracardiac diseases. No early deaths occurred in group 1, compared with 1 infant (4%) in group 2. Major complications occurred similarly in both groups (9% versus 12%). There were two late deaths in group 1 (9%) and none in group 2. No surviving patients have required a second ventricular septal defect operation, and the majority no longer receive anticongestive therapies. These results indicate that primary surgical closure of large ventricular septal defects, even multiple muscular defects, can be performed in very small infants with no difference in mortality or serious complication rates compared with larger infants. Protracted medical efforts to achieve larger size before primary repair and palliative pulmonary artery banding are not necessary.

Successful management of congenital atrioventricular block associated with hydrops fetalis

nal contents do not oppose the most efficient posterior portion of the diaphragm. We have also shown that the improvement with prone positioning was transient and could be reversed by repositioning the patient into the supine position. This finding testifies to the instability of the positional pulmonary relationships in neonates. Prone positioning should be used in the postextubation period for as long as possible to prevent this reversal of beneficial effects. We recommend that, in the immediate postextubation period, infants with respiratory distress syndrome be cared for while they are in the prone position.

Intraoperative Evaluation of Atrioventricular Septal Defect Repair by Color Flow Mapping Echocardiography

Despite improving survival rates after repair of atrioventricular septal defect, many patients require reoperation because of postoperative heart failure. We used intraoperative color flow mapping echocardiography to assess the results of surgical repair of atrioventricular septal defect in 19 consecutive patients and compared those findings with results three to five days and 3 to 11 months after repair. There was close correlation between intraoperative and postoperative color flow mapping echocardiography in estimating the presence and severity of left atrioventricular valve regurgitation. All patients survived surgical repair, but in 4 (21%), postoperative congestive heart failure due to left atrioventricular valve regurgitation developed. The need for reoperation was significantly correlated with the severity of left atrioventricular valve regurgitation (r = 0.68) as estimated by intraoperative echocardiography and preoperative aberrancies in the atrioventricular valve (r = 0.68). Age, weight, additional congenital heart disease, preoperative pulmonary vascular resistance, preoperative atrioventricular valve regurgitation, and postoperative mean pulmonary arterial or left atrial pressure were not significantly correlated with the need for reoperation. Intraoperative color flow mapping echocardiography can accurately predict the development of early postoperative heart failure and subsequent reoperation after surgical repair of atrioventricular septal defect.

Diagnosis of anomalous left coronary artery from the pulmonary trunk by color Doppler echocardiography

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