Soraya Nouri

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

Long-Term Follow-Up Results of Balloon Angioplasty of Postoperative Aortic Recoarctation

Immediate- and short-term follow-up results of balloon dilatation of aortic recoarctation following surgery have been well documented, but there is sparse data on long-term follow-up. During a 10-year period ending in August 1995, 33 children, aged 2 months to 14 years old, underwent balloon angioplasty of aortic recoarctation. Prior surgery included resection and end-to-end anastomosis (n = 9), subclavian flap (n = 16) or prosthetic (Dacron or Gore-Tex) patch (n = 5) angioplasty, and repair of an interrupted aortic arch (n = 3). Recoarctation developed 1 month to 14 years (mean +/- SD 29 +/- 44 months) after surgery. The indications for angioplasty were peak-to-peak systolic gradients > 20 mm Hg and systemic hypertension and/or congestive heart failure. After balloon angioplasty, the peak-to-peak systolic pressure gradient across the coarctation decreased from 48 +/- 22 to 13 +/- 15 mm Hg (p <0.01), and the size of the coarcted segment increased from 3.3 +/- 1.4 to 6.5 +/- 2.3 mm (p <0.01). Follow-up angiography and/or magnetic resonance imaging were performed in 20 children 17 +/- 12 months after angioplasty. No aneurysms were observed and improvement in the diameter of the coarcted aortic segment (9 +/- 3 mm) persisted. One- to 10-year (median 5) clinical follow-up was available in 32 children. During follow-up, 2 children required surgery to repair a long tubular isthmic narrowing. The residual gradients, determined by arm-leg systolic blood pressure difference, were 5 +/- 8 mm Hg. No patient was symptomatic and only 1 patient (3%) was hypertensive, controlled with antihypertensive medications. We conclude that balloon angioplasty of aortic recoarctation following all types of surgical repair is feasible, safe, and effective with good long-term results. We recommend balloon angioplasty as the procedure of choice in the management of postsurgical recoarctation with hypertension and/or congestive heart failure.

Glenn Shunt: Long-Term Results and Current Role in Congenital Heart Operations

Fifty cyanotic patients (aged 2 days to 22 years) underwent Glenn shunts for tricuspid atresia and other cyanotic heart defects. Thirteen of 15 operative deaths occurred in infants less than 4 months old, and only 1 death has occurred in the last 9 years. Results were poor in patients with Ebsteins anomaly, truncus arteriosus, transposition of the great vessels, and complex defects other than tricuspid atresia and univentricular heart. Of the 35 patients followed from 0.9 to 14.8 years, 12 were followed for more than 10 years. None of the 11 late deaths could be attributed to complications of the shunt. Minimal evidence of intrapulmonary shunting was found by angiography, pulmonary venous oximetry, or radioisotopic studies. Late deterioration due to venous collaterals and decreased flow to the opposite lung necessitated Blalock-Taussig shunts in 6 and Fontan procedures in 10. All survived the Fontan procedures with minimal morbidity. These data support the concept that Glenn shunts do not necessarily result in pulmonary abnormalities and may be indicated as a staged procedure in a few selected patients prior to a Fontan procedure.

Detection of coronary artery abnormalities in tetralogy of Fallot by two-dimensional echocardiography.

Patients with tetralogy of Fallot have a 5% to 19% incidence rate of abnormal distribution of coronary arteries. These abnormalities are usually detected by angiography and influence the timing and mortality rate of surgery. This study evaluates two-dimensional echocardiography as a method of assessing coronary artery distribution in tetralogy of Fallot. Forty-five consecutive patients with tetralogy of Fallot, aged 0.1 to 20.5 years (mean 5.7 +/- 4.3), had prospective two-dimensional echocardiographic studies to examine the branching patterns of the coronary arteries and to determine the presence or absence of a branch from the right or left coronary artery that crossed the right ventricular outflow tract. The first two patients had known coronary abnormalities and served as learning models. All other echocardiographic studies were performed without knowledge of angiographic or surgical findings. Twenty-two studies were completed before coronary angiography (group A) and 23 after angiography (group B). All eight patients (18%) with coronary abnormalities were correctly identified by two-dimensional echocardiography (five in group A and three in group B). Three had bilateral anterior descending coronary arteries originating from the left and right coronary arteries, two had the anterior descending artery originating from the right coronary artery, two had a large conal branch from the right coronary artery and one had origin of both left and right coronary arteries from a single left ostium. All abnormal coronary arteries were visualized crossing the right ventricular outflow tract, whereas all 21 small conal branches from the right coronary artery were not seen in the right ventricular outflow tract.(ABSTRACT TRUNCATED AT 250 WORDS)

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.

Aortico-right ventricular tunnel and critical pulmonary stenosis: Diagnosis by two-dimensional and Doppler echocardiography and angiography

SummaryAn infant with aortico-right ventricular (AO-RV) tunnel and critical pulmonary stenosis presented with severe distress at birth. We present the clinical, echocardiographic, and angiographic features, correlated with autopsy findings. Also we discuss the differentiation from other AO-RV communications and a theory for the embryogenesis. AO-RV tunnel should be considered in the differential diagnosis of a critically sick newborn with cyanosis, a “to and fro” murmur, and signs of right heart failure. The correct diagnosis can be made echocardiographically by demonstrating the two ends of the tunnel connecting the aorta and a dilated RV, two normal coronary arteries, and obtaining high-velocity systolic and diastolic Doppler flow signals in the tunnel. Surgical repair of this lesion is possible, and early diagnosis and a modification of the surgical procedure may help survival.

Functional pulmonary atresia: Color flow recognition and treatment with extracorporeal membrane oxygenation*

diographic, and echocardiographic evidence of a diffuse process tha t involved the h e a r t J Autopsy of the hear t in question revealed a lymphocytic and eosinophilic infi l trate with prominent areas of myocardial necrosis. Our pat ient , however, did not show either enzymatic or echocardiographic evidence of active myocarditis. I t is possible tha t the cells tha t par t ic ipate in the inflammatory reaction could have a predeliction for the conduction system but autopsy confirmation is lacking. On the basis of this case and others in the l i terature, we believe tha t cardiac complicat ions of IL-2 are common and advocate a careful assessment of myocardial function and screening for underlying coronary ar tery disease. We recommend noninvasive imaging techniques such as echocardiography or radionuclide angiography and treadmil l exercise testing. Selected persons should undergo coronary angiography. Even with these precautions, bradyarrhythmias and tachyarrhythmias can occur unpredictably, and continuous monitoring is strongly recommended for in these persons.

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)

Repair of Aortic Coarctation in Infants: Experience with an Intraluminal Shunt

From 1962 to mid-1984, 63 infants underwent coarctation repair. Cardiac defects were present in 46 (73%). Repair was by subclavian aortoplasty in 35 patients, resection and end-to-end anastomosis in 19, and other techniques in 6. Three patients died before the repair was completed. In 15 patients, an intraluminal shunt was used during subclavian aortoplasty. Prostaglandin E1 (PGE1) was infused in 9 patients. Early (thirty-day) mortality was 16% (10 patients): 4 patients who underwent end-to-end anastomosis; 3 during attempted end-to-end anastomosis; 2 who received bypass grafts; and 1 who had subclavian aortoplasty without a shunt. None of the 15 patients who had subclavian aortoplasty with a shunt died. There were no early deaths among the last 25 patients seen. One patient who underwent subclavian aortoplasty without a shunt is paraplegic. There were 10 late deaths among the 53 patients followed from 1 month to 12 years (mean, 3 years). Arm-leg pressure gradients of 20 mm Hg or greater were found in 4 of the patients who had end-to-end anastomosis but not in any of the patients who had subclavian aortoplasty. Improved results of coarctation repair in infants in this study were attributed to PGE1, subclavian aortoplasty, and use of an intraluminal shunt.