Zia Q. Farooki

Spontaneous regression of cardiac rhabdomyoma

Abstract Multiple cardiac rhabdomyomas in a neonate with tuberous sclerosis were first described by Von Recklinghausen in 1862. These hamartomas are the cardiac tumors most frequently encountered during infancy and childhood. Rhabdomyomas account for 45% of primary heart tumors in children and represent 53% of primary benign childhood cardiac tumors. 1 Approximately 30% of patients with tuberous sclerosis have cardiac rhabdomyomas. 2 Their natural history is unclear because most reviews on this subject are based on autopsy data. The prognosis for cardiac rhabdomyomas is believed to be grim because of reported fatality rates of 53% by the first week of life and 78% by 1 year of age. 2,3 With widespread use of echocardiography in pediatrics during the last 2 decades, it has become clear that rhabdomyomas result in a wide spectrum of clinical manifestations, ranging from a total absence of symptoms to intrauterine or sudden postnatal death. Also reported are hydrops fetalis, dysrhythmias, inflow or outflow obstruction, congestive heart failure and possibly cerebral embolization. Histologic examination of these masses in 1923 was suggestive of spontaneous regression. 4 Isolated clinical reports of spontaneous regression have recently appeared. 5 We now describe a series of 5 infants with tuberous sclerosis who had close documentation of the size of their 13 tumors.

Ultrasonic Pattern of Ventricular Rhabdomyoma in Two Infants

Abstract Two infants with ventricular septal rhabdomyoma were studied with ultrasound. An abnormal cluster of echoes in the vicinity of the ventricular septum corresponded to the angiographic location of the tumor in each case. Disappearance of these echoes after successful surgical resection lends further support to the conclusion that the abnormal cluster of echoes was indeed related to the intracavitary portion of the tumor in both cases.

Congenital coronary artery fistulae: a review of 18 cases with special emphasis on spontaneous closure.

SummaryBetween 1972 and 1990, 18 patients (median age 3 years, range 0.1–14 years) with coronary artery fistulae (CAF) were seen at this institution. Of the 16 patients without associated heart defects, two patients presented with congestive heart failure and the remaining 14 were asymptomatic. Fifteen patients had normal orgin of two coronary arteries. Two patients has atresia of the proximal right coronary artery and, in one patient, the right coronary artery originated from the left main coronary artery. The QP/QS ranged between 1.0 and 2.8, with a mean of 1.4. Fifteen patients underwent operative closure without any deaths. One patient is being followed medically at present. Two patients showed spontaneous clinical improvement of CAF (complete closure in one and near complete in the other) and remained asymptomatic during a decade of follow-up. Review of the literature suggests operative closure of symptomatic and asymptomatic small CAF during childhood. However, the natural history of minute CAF remains unclear. In addition, symptoms from CAF may spontaneously improve with time.

Augmented norepinephrine and renin output in response to maximal exercise in hypertensive coarctectomy patients

To evaluate a possible neural or renal contribution to the hypertension that occurs in some patients following coarctation of aorta repair, 35 patients underwent graded bicycle exercise with serial measurements of plasma norepinephrine concentrations and plasma renin activity. Sixteen patients with coarctectomy who had systolic or diastolic hypertension at peak exercise were compared with 19 normotensive patients with coarctectomy. The average time interval between coarctation repair and study was significantly longer (p less than 0.05) in the hypertensive group than in the normotensive patients (12.8 +/- 4.8 versus 8.7 +/- 2.2 years). The heart rate response to exercise was similar for both patient groups. The systolic blood pressure in the hypertensive group was higher than in the normotensive group at rest in the supine and upright positions and at 5 minutes of recovery, in addition to peak exercise, and the diastolic blood pressure was increased at peak exercise. Plasma norepinephrine concentrations were significantly higher at peak exercise and during recovery in the hypertensive group than in the normotensive patients. Plasma renin activity was also significantly higher in the hypertensive group at peak exercise. These data suggest that patients with coarctectomy who have a hypertensive response to exercise have an augmented sympathetic nervous system output and increased plasma renin activity that may lead to peripheral vasoconstriction at peak exercise and that may contribute to the development of their hypertension.

Echocardiographic spectrum of Ebstein's anomaly of the tricuspid valve.

Sixteen patients aged between one day to 18 years with Ebsteins malformation of the tricuspid valve were studied with ultrasound. The findings were compared with a group of 74 patients without Ebsteins malformation. Two features were considered specific for Ebsteins malformation: 1) ability to record the anterior tricuspid leaflet (ATL) farther to the left of the left sternal border than in the control group; and 2) abnormally prolonged interval between the “C” points of the tricuspid and the mitral valve echoes (M, T, I). All other parameters measured were nonspecific for Ebsteins anomaly of the tricuspid valve. The sail sound was recorded in ten patients with Ebsteins malformation and occurred at the time when the anterior tricuspid leaflet was in the most posterior position.In a patient with congenital heart disease, an M, T, interval greater than 0.03 sec and recording of an anterior tricuspid leaflet near the apex of the heart strongly suggest the diagnosis of Ebsteins malformation. These two criteria were not fulfilled in any patient who did not have Ebsteins malformation. Conversely, however, absence of these two features does not rule out Ebsteins anomaly of the tricuspid valve.

Pacing in children and young adults with nonsurgical atrioventricular block: Comparison of single-rate ventricular and dual-chamber modes

A prospective comparison of physiologic response to single-rate ventricular and dual-chamber atrioventricular pacing was conducted in 14 pediatric patients (age 1 to 24 years, median 14) with symptomatic nonsurgical second- or third-degree atrioventricular block. All patients were studied acutely during cardiac catheterization before and after 1 hour of both pacing modes. Following pacemaker implant, eight patients were reevaluated after 1 month of each mode with symptom questionnaire, resting ECG, resting echocardiogram, and Doppler cardiac output measurement at rest and at peak treadmill exercise. Cardiac outputs (mean +/- standard error) increased acutely (n = 14) with both ventricular (32 +/- 12%) and dual-chamber (39 +/- 10%) pacing over intrinsic rhythm values (p less than 0.01 in both). During chronic pacing (n = 8), symptoms were reported only with the ventricular mode. Dual-chamber synchronous pacing was associated with improved mean resting shortening fraction and cardiac output, slower mean resting sinus rate (89 +/- 5 compared to 73 +/- 4 bpm (p less than 0.02), and a 23% increase in mean excerise cardiac output (4.2 +/- 0.4 compared to 3.4 +/- 0.3 L/min/m2) compared to single-rate ventricular pacing. Exercise-induced dysrhythmias occurred only with ventricular pacing. This study demonstrates that pediatric patients with nonsurgical atrioventricular block can compensate for loss of atrioventricular synchrony at rest but exhibit improved cardiac function with chronic dual-chamber atrioventricular compared to single-rate ventricular pacing.

Surgical and medical results of complete atrioventricular canal: A ten year review

The files of 121 patients who presented to Childrens Hospital of Michigan over the last 10 years with complete atrioventricular (AV) canal were reviewed to evaluate long-term management and overall outcome. Of 121 patients, 70 underwent corrective surgery, 21 (30%) of whom died perioperatively. The surgical mortality rate was 13% when patients with hypoplastic left or right ventricle (n = 6), double-orifice mitral valve or extreme deficiency of mitral tissue (n = 5), and pulmonary vascular obstructive disease (n = 5) were excluded. Of the 49 patients who survived operation, 36 are in New York Heart Association class I, 1 patient requires a pacemaker and 3 died late. In 34 of the 51 patients (28%) who did not undergo operation, pulmonary vascular obstructive disease developed; it occurred within 12 months in 10 patients (8%). Eight other patients who did not undergo operation died before planned surgery (age 1 to 9 months). Although surgical prognosis in good candidates is acceptable, the overall prognosis for children with complete AV canal is guarded because of the risk of early death or early pulmonary vascular obstructive disease and frequently unfavorable anatomy.

Echocardiographic differentiation of a cystic and a solid tumor of the heart.

A hydatid cyst in the ventricular septum was diagnosed with echocardiography in a child with abdominal echinococcosis. The finding was confirmed with cardiac catheterization, cineangiography and surgical removal and pathologic examination of the cyst. The echocardiographic features of the fluid-filled hydatid cyst are contrasted with those of solid left ventricular tumor (rhabdomyoma) in a newborn.

Hepatic irradiation and adriamycin cardiotoxicity

mediated immuni ty is one of the important determinants in the hosts defense against P. carinii. The present report emphasizes that children with congenital hypogammaglobul inemia are at risk for P. carinii pneumonia and that this may be the initial infection which brings these children to medical attention. This organism must therefore be considered, along with the usual bacterial pathogens, in a child with hypogammaglobulinemia who presents with his first pneumonia. These children are also at risk for recurrent P. carinii pneumonia. Since tr imeth0prim-sulfamethoxazole prophylaxis has been shown to be effective in preventing P. carinii pneumonia in cancer patients, 3 consideration should be given to the use of this drug for prophylaxis in children wi,h hypogammaglobul inemia who have recovered from an episode of P. carinii pneumonia.

Primary pulmonary artery sarcoma in two children

SummaryThe clinical and pathological features of primary pulmonary artery sarcoma in two children are reported. The first patient presented with right ventricular outflow obstruction and underwent successful surgical resection of his tumor. The second patient developed cardiac arrest following a relatively short period of symptoms of right heart failure and could not be revived. The pathological diagnosis in both patients was hemangiopericytoma. To the best of our knowledge, primary pulmonary artery sarcoma in children has not been previously reported.