Matthew Farina

Clinical presentation of rhabdomyoma of the heart in infancy and childhood

Abstract Four infants with cardiac rhabdomyomas were seen during the past 2 years at Albany Medical Center Hospital; 2 were siblings. The presenting cardiac problems in the 4 infants were congestive heart failure, cyanosis secondary to shunt reversal at atrial level, paroxysmal atrial tachycardia and significant subaortic stenosis, respectively. All 4 patients had tuberous sclerosis. Surgical removal of the tumor was performed in 2. The signs and symptoms produced by myocardial dysfunction secondary to the tumor are reviewed. It is pointed out that the cardiac symptoms produced by rhabdomyoma are not specific and can be produced by other types of cardiac tumors. The possibility of having a second child with rhabdomyoma should not be dismissed. Surgical removal of the tumor should be considered when it is producing significant obstruction of the blood flow on either side of the heart.

Clinical Aspects of Congenital Heart Disease in Mongolism

Abstract The clinical course of 39 children with mongolism and congenital heart disease is presented. In all the diagnosis was confirmed by cardiac catheterization, angiocardiography and chromosomal studies. Patients were classified into 3 groups according to the hemodynamic findings as follows: group I, 6 patients with right ventricular outflow tract obstruction; group II, 22 patients with pulmonary hypertension at systemic levels; and group III, 11 patients with normal or slightly increased pulmonary vascular resistance. The underlying cardiac pathologic findings in these 3 groups are presented. In group I, the mortality rate was 33 percent, and hypoxia was the major problem. Recurrent chest infections and congestive heart failure developed in patients in group II, and the mortality rate was 36.5 percent. Eisenmengers reaction developed by age 3 years in the majority in this group. Patients in group III did extremely well, and most were asymptomatic.

Cardiac Rhabdomyoma in the Neonate: Surgical Management

Rhabdomyoma is the most common cardiac neoplasm in neonates. Tuberous sclerosis is found in half of the patients with rhabdomyomas. We maintain a surgical policy of accepting for operation only neonates in whom it has been demonstrated that the primary cause for hemodynamic compromise is obstructing, intracavitary neoplasms. Only the intracavitary portions of the rhabdomyoma are excised; no effort is made to completely remove all intramural tumors. Rhabdomyomas demonstrate benign pathological characteristics and may regress. Neonates with rhabdomyomas but no hemodynamic impairment, or those in whom only intramural masses can be demonstrated, are not considered surgical candidates. Tuberous sclerosis by itself should not be judged a contraindication to operation. The results of our surgical policy regarding rhabdomyomas in neonates are reported in two case presentations.

Left ventricular rhabdomyoma causing subaortic stenosis—the two-dimensional echocardiographic appearance

SummaryWe report the preoperative and postoperative clinical findings, M-mode and two-dimensional echocardiograms, and the cardiac catheterization findings in a newborn with left ventricular rhabdomyoma causing severe subaortic stenosis. The tumor was diagnosed by echocardiography at 3 hours of age. The clinical implications of the case are discussed.

Right Heart Reconstruction Following Repair of Tetralogy of Fallot

Ten patients in whom tetralogy of Fallot had been repaired underwent late reconstruction of the outflow tract of the right ventricle because of poor hemodynamic results. The major hemodynamic problems that necessitated right ventricular (RV) outflow tract reconstruction were severe pulmonary insufficiency in 9 patients and pulmonary stenosis in 1. Impaired RV contractility and RV aneurysm were the most important factors prompting valve replacement for severe pulmonary insufficiency. Seven patients received a Hancock prosthesis and 3, an aortic homograft. Among the 7 patients who underwent postoperative cardiac catheterization, the surgical results were hemodynamically excellent in 2, good in 3, and unsatisfactory in 2. The management of pulmonary insufficiency in such patients is discussed.

Congenital pulmonary and subclavian arteries steal syndrome

Abstract A 2-month-old infant was seen at Albany Medical Center Hospital with right peripheral facial palsy and dysphagia. Evaluation of the cardiovascular system showed a lack of left common carotid and left subclavian pulses. A continuous murmur was heard to the left of the sternum. Cardiac catheterization and angiography demonstrated retrograde filling of the left common carotid artery, the left subclavian artery, a left dorsal aorta, and the pulmonary artery from the opposite side of the cerebral hemisphere. Autopsy revealed a right aortic arch and a right thoracic aorta. The right common carotid and right subclavian arteries arose from the right aortic arch. A large left dorsal aorta arose from the pulmonary artery and divided superiorly into the left common carotid and the left subclavian arteries. There was no communication between the two aortae. The central nervous system signs were thought not to be secondary to pulmonary artery steal. It was suggested that a lack of pulses and a continuous murmur would suggest such a diagnosis.

Unexpected resistance to external cardiac compression may signal pericardial tamponade.

Pericardial tamponade associated with central catheters is often lethal, unless promptly identified. We report our experience with two infants who suffered this complication but were successfully resuscitated. In each case, associated radiographs showed the tip of the central catheter beyond the superior vena cava–right atrium (SVC–RA) junction. Identifying the clinical triad of (1) sudden cardiovascular collapse unresponsive to usual resuscitative measures, (2) thoracic transillumination not suggestive of air leak, and (3) unexpected resistance to external cardiac compression led to the working diagnosis of pericardial tamponade and therapeutic pericardiocentesis. Prompt recovery followed. Central catheters within the RA seem prone to cause pericardial tamponade.

Cardiologic assessment in participants of Pop Warner Junior League football

Physical examinations and electrocardiograms were performed on 50 young men in preseason training for Pop Warner Foot ball. Functional murmurs were found in 52%. The majority of electrocardiographic findings were within the accepted normal range for nonathletes but some were consistent with findings in trained athletes. Awareness of these normal variations is im portant for proper assessment of prospective and participating athletes.

Opacification of the Right Heart Complicating Left Ventricular Angiography

Abstract In the case presented, opacification of the coronary sinus and right heart occurred immediately after extravasation of contrast medium into the left ventricular myocardium. Following angiography, electrocardiogram and vectorcardiogram changes were noted. The patient suffered no sequelae. When this complication occurs it may mimic intracardiac shunts.