Su-chiung Chen

Clinical profile of conjestive cardiomyopathy in children

Abstract The clinical profile of 23 children with congestive cardiomyopathy was reviewed to detect any factors that might be predictive for their survival. Factors examined include age at onset ( 2 years), geader, severity of the clinical picture including data from the chest radiograph electrocardiogram (ECG), echocardiogram, hemedynamic study and endomyocardial biopsy. Follow-up study ranged from 1 month to 14 years (mean 43 months). There were 12 survivors and 11 nonsurvivors; the 1 year mortality rate was 30% (7 of 23), and the 5 year mortality rate was 44% (10 of 23). Age at onset, gender, cardiothoracic ratio on chest radiograph, pattern of infarction, ST-T changes or arrhythmia on ECG and left ventricular enddiastolic pressure were nonpredictive of outcome. However, low shortening fraction (mean 11.5% in nonsurvivors versus 20.9% in survivors, p

Clinical spectrum of restrictive cardiomyopathy in children

We reviewed the clinical spectrum and possible prognostic factors in 14 children with restrictive cardiomyopathy. The patients were not homogeneous in clinical presentation or morphology. The mortality rate was high: 21.4% at 1 year and 50% at 2 years after presentation. Younger patients with respiratory symptoms, thromboembolism, increased cardiothoracic ratio on chest radiogram or patients with endocardial fibroelastosis appear to have a worse prognosis and orthotopic cardiac transplantation may be indicated.

Familial syndrome of progressive arterial occlusive disease consistent with fibromuscular dysplasia, hypertension, congenital cardiac defects, bone fragility, brachysyndactyly, and learning disabilities

We report on 4 of 9 sibs with a syndrome of stenosis of the renal arteries and chronic hypertension, variable stenosis or occlusion of cerebral, abdominal and probably coronary arteries due to suspected fibromuscular dysplasia, congenital cardiac abnormalities, brachydactyly and syndactyly of the hands and feet, and increased bone fragility consistent with a mild form of osteogenesis imperfecta. Three affected individuals have had mild to moderate learning disabilities. The parents and the remaining 5 sibs have normal hands and feet and no history of excessive fractures. Individual components of this syndrome may appear as isolated conditions, including fibromuscular dysplasia, brachydactyly, syndactyly, and osteogenesis imperfecta, and are autosomal dominant traits in many cases. Explanations for this familial occurrence include autosomal recessive inheritance, autosomal dominant inheritance with decreased penetrance, or parental gonadal mosaicism for a mutation involving a single gene or several contiguous genes.

Transthoracic Doppler Echocardiography of Normally Originating Coronary Arteries in Children

Transthoracic Doppler color flow and spectral velocity patterns of normal coronary arteries in children have not been well studied. We designed this study to evaluate coronary artery flow velocity characteristics in normal and hypertrophied hearts. Sixty-eight children with optimal two-dimensional echocardiographic images of the left coronary artery (LCA) and right coronary artery (RCA) were prospectively studied. The heart was normal in 45 children, and 23 had left and/or right ventricular hypertrophy assessed by echocardiography (mean age 5.8 versus 5.2 years, p = NS). Color flow signals were detected in the LCA in 63(92%) of the 68 children studied, and pulsed Doppler spectral waveforms were recorded in 47 (69%). The latter were recorded in 26 (58%) of 45 normal children and in 21 (91%) of 23 children with left ventricular hypertrophy. Diastolic RCA flow signals were detected mostly in those with right ventricular hypertrophy (10 of 10). Higher levels of LCA maximum diastolic velocity (42 +/- 23 versus 24 +/- 6 cm/sec, p = 0.0004), increased diastolic flow (16 +/- 15 versus 6 +/- 4 ml/min, p = 0.01), and delayed time to peak diastolic velocity expressed as a percentage of diastolic spectral duration (38% +/- 14% versus 20% +/- 8%, p = 0.0001) were observed in children with left ventricular hypertrophy than in those in normal children. A strong correlation was present between Doppler-derived LCA flow and left ventricular mass/m2 (r = 0.7, p = 0.001). In normal hearts, LCA spectral velocity pattern did not change with increasing age, but the time velocity integral became progressively larger, resulting in a strong correlation with weight (p < 0.001, r = 0.78). This study demonstrates (1) LCA flow signals can be detected and quantitated in the majority of children with and those without left ventricular hypertrophy. (2) Left ventricular hypertrophy is associated with increased LCA flow, higher diastolic velocity, and delayed peak diastolic velocity. (3) RCA flow signals are mostly detected when there is right ventricular hypertrophy. Studies on larger groups of patients are needed to further confirm our observations and to enhance understanding of coronary artery flow reserve.

Dysrhythmias after the modified Fontan procedure

SummaryThe cardiac rhythm before and after the modified Fontan procedure was reviewed in 24 patients. Transient atrial dysrhythmias were common in the immediate postoperative period. Late postoperative premature atrial contractions were detected by ambulatory monitoring in 20 of 23 patients; eight (34.8%) had supraventricular tachycardia. Late ventricular dysrhythmia was detected in 18 of 23 patients: ten had low-grade ventricular dysrhythmias and eight (34.8%) had multiform premature ventricular contractions. Five of the latter had couplets and one of these five plus another had ventricular tachycardia. Seven patients with supraventricular tachycardia and five patients with ventricular dysrhythmia required antiarrhythmic medication. Asymptomatic bradycardia was detected in five patients (21.7%). One patient had intermittent second-degree atrioventricular block. No specific risk factors predicted dysrhythmias. Thus, cardiac dysrhythmias were common in patients after the modified Fontan procedure, but were well tolerated in most patients. No sudden deaths or syncopal episodes have occurred during a mean follow-up of 5 years. One patients death was related to severe left ventricular dysfunction. Permanent pacing has not been required in any patient.

Concomitant Valvotomy and Subclavian–Main Pulmonary Artery Shunt in Neonates with Pulmonary Atresia and Intact Ventricular Septum

Our current approach to the management of neonates with pulmonary atresia and intact ventricular septum is to perform a transarterial pulmonary valvotomy through a left anterolateral thoracotomy followed by a polytetrafluoroethylene shunt between the left subclavian artery and the pulmonary trunk at the site of the pulmonary arteriotomy. From October, 1983, to December, 1985, 7 consecutive neonates with pulmonary atresia and intact ventricular septum were managed in this fashion. Mean age was 5.1 days (5 patients, less than 48 hours old), and mean weight was 3.3 kg (range, 2.5-4.3 kg). Right ventricular morphology was type I (tripartite) in 4 patients, type II (absent trabecular portion) in 2, and type III (absent trabecular and infundibular portions) in 1. The mean right ventricular to left ventricular peak systolic pressure ratio was 1.5. One patient who initially had valvotomy alone required a left subclavian-pulmonary trunk shunt the next day for hypoxemia. All other patients had a valvotomy and shunt during the same procedure. There were no operative or hospital deaths. Follow-up of 3.5 to 34 months (mean, 17.5 months) confirmed shunt patency in all patients. Three of 4 patients undergoing postoperative catheterization have shown good right ventricular growth; 2 have undergone successful repair at 10 and 23 months. There have been 3 late deaths at 3.5, 4, and 8 months. Two other patients are doing well and are awaiting postoperative catheterization. This procedure permits synchronous valvotomy and shunting without the need for cardiopulmonary bypass in these critically ill neonates.(ABSTRACT TRUNCATED AT 250 WORDS)

Fatal Aortic Rupture Presenting as Chest Pain in an Adolescent: The Role of Echocardiography in Occult Cystic Medial Necrosis

An adolescent female with occult cystic medial necrosis died following spon taneous aortic rupture. A large saccular aortic aneurysm that had ruptured into the pericardial space was demonstrated by two-dimensional echocardiography and confirmed at surgery. Echocardiographic screening of the patients family members revealed a 13-year-old brother with unsuspected aortic root dilatation. He is now being followed for possible progression of his disease. This case demonstrates the role of echocardiography in cystic medial necrosis. It can aid the acute management of patients with aortic dissection or aneurysm. It can also define patients with occult disease who require serial follow-up and genetic counseling.

Transient Hypertrophic Cardiomyopathy in Neonates

Vaillant et al. [1] reported three neonates with fetal distress who developed transient hypertrophic cardiomyopathy and they postulated myocardial ischemia as the cause of hypertrophy. A neonate at our institution, with presumed sepsis and disseminated intravascular coagulation, developed hypertrophic cardiomyopathy, and his endomyocardial biopsy findings support the ischemic hypothesis. A 36-week gestational age male was delivered vaginally 6 hours after spontaneous rupture of membrane. Birth weight was 2.67 kg. He became cyanotic shortly after birth. Arterial blood gas showed pH was 7.22, pCO2 was 44 mmHg, and PO2 was 54 mmHg. He was given ampicillin and gentamycin and was intubated. WBC was 14,300/mm 3 , hemoglobin was 16.8 g/dl, hematocrit was 50.4%, and platelet was 351 × 10 3 /mm 3 . Blood glucose was 20 mg/dl. Cerebrospinal fluid contained 7 lymphocytes, 28 mg/dl glucose, and 97 mg/dl protein. No organism was seen on smear. LDH was 1450 IU/L and CPK was 55 IU/L. Chest x-ray showed bilateral infiltrates with air bronchogram. Repeat WBC 4 hours later was 4700/mm 3 with left shift. Platelet count was 3000/ mm 3 . Prothrombin time was 32.9 seconds, PTT 35 seconds, fibrinogen was 160 mg%, fibrin degradation product was >10, <40 g/ml. Bacterial and viral cultures from cerebrospinal fluid, blood, nasopharynx, and rectum were all negative. He received platelet and fresh frozen plasma infusion. An electrocardiogram (ECG) on day 3 showed right atrial enlargement and low left ventricular voltage. Echocardiogram that day showed normal left ventricle (LV) wall thickness and a decreased LV shortening fraction of 18% and digoxin and Lasix were started. He also developed hemoglobinuria and impaired renal function. Abdominal ultrasound showed a mass at aortic bifurcation and an echogenic right kidney. Renal scan showed nonperfused right kidney on day 8. Blood pressure gradually increased from the fourth day and reached 132 mmHg systolic on day 10 and he was treated with hydralazine and captopril. Repeat echocardiogram at day 7 continued to show LV dysfunction, but on day 18 it showed marked changes with thick right ventricular wall and ventricular septum (7 mm in diastole) and concentric LVH. A small mass was seen in the right atrium and thrombus was suspected. Digoxin was discontinued. He had an endomyocardial biopsy on day 19. Pathologic examination showed thickened endocardium with marked endothelial proliferation. The subendocardial muscle fiber showed degeneration and necrosis with multiple foci of interstitial fibrosis. The blood vessels showed extensive endothelial cell proliferation. The findings were consistent with ischemic changes. The patient improved gradually and was discharged at 1 month without medication. Follow-up at 2 months showed improved biventricular hypertrophy and resolved right atrial clot. At 1 year, echocardiogram and ECG were normal. As the authors indicated, transient myocardial hypertrophy in perinatal distress could be related to increased catecholamines or adaptation to pressure or volume overload. However, the myocardial histology in the present case is consistent with myocardial ischemia.

741 A DEMORAPHIC STUDY OF TRISOMY 13

Trisomy 13 occurs with an incidence of 1/8000 live births. Thirty-one patients with Trisomy 13 diagnosed over the past 19 years at our institutions were reviewed. Parameters evaluated were age of parents, sex, occupation, prenatal history, parity, family history, geographic area, clinical features, and birth month. 23/31 (74%) were male; three periods of the year with higher incidence (28/31) were found (March-April, July-August, and November-December); mean age of mother was 29.6 years (median 28 years). Trisomy 13 in our patients was associated with increased parity. Family history revealed 2 patients who had relatives with Trisomy 21; 2 patients had relatives with polydactyly; 2 patients had relatives with cleft lip/palate. These data refute previous data which indicate a sex ratio in Trisomy 13 of 1:1 and 2 maternal age peaks. In addition, the data support a higher incidence in males, 3 peak months of occurrence, an increased risk with increased parity. The occurrence in the family of cleft lip/palate and polydactyly, which are characteristic features of Trisomy 13 syndrome, as well as other trisomies suggests that there may be other genetic influences affecting non-disjunction in families.

Persistent right valve of the sinus venosus presenting as a right atrial cystic mass

A boy was born at 31 weeks of gestation to a mother with a history of alcohol and drug abuse. The birth weight was 1 kg and the infant had intrauterine growth retardation. He had a protracted stay in the hospital for respiratory distress, chronic lung disease, and ventilator dependency and had multiple percutaneous indwelling catheters at several sites. At 5 months, he had fever and coagulase-negative staphylococcus aureus was cultured from his blood. A transthoracic echocardiogram at this time revealed a right atrial mobile, cystic, pedunculated mass extending into the right ventricle through the tricuspid valve (Fig. 1). This was interpreted as a possible vegetation, thrombus, or cor triatriatum dexter, and he was started on vancomycin and cefotaxime. He died at 8 months after progressive respiratory insufficiency. At autopsy, examination of the heart revealed a large, redundant, parachute-like membranous sac (length, 1.4 cm) in the right atrial chamber with attachment by thin fibrous bands to the atrial wall near the inferior vena caval orifice and to the limbus of fossa ovalis (Fig. 2). Other than a patent foramen ovale, the heart, including the tricuspid and pulmonary valves, was normal. Discussion