In order to help determine the best method of care for patients with complex or atypical congenital or acquired heart conditions, a Clinical Management Conference is held regularly at Children's.

Open to all fellows, medical staff, nurses, and technicians in the Cardiovascular Program as well as to other Children's staff and visiting professionals, the conference is an opportunity for a clinician to obtain multiple opinions regarding the care of a patient who is clinically challenging. This feedback helps clinicians provide the best possible care and gives families the assurance that many experienced physicians have reviewed their child's cardiac status. The conference also provides primary cardiologists a well-reviewed second opinion and their participation in the presentations and discussion at the conference is an important contribution.

Two to three patients are reviewed during each conference. The primary cardiologist presents a clinical summary, pertinent laboratory data are analyzed, and recent literature is summarized. Two illustrative cases from recent conferences are discussed below.

Case 1: Atypical coarctation of the aorta in a 14-year-old boy

At 2 years of age, this patient was noted to have a blood pressure systolic gradient from his right arm to right leg. A cardiac catheterization demonstrated coarctation of the transverse aorta with a 25 mm Hg gradient, hypoplasia of the left carotid and left subclavian arteries, and tortuosity of the distal transverse aorta and proximal descending thoracic aorta. A follow-up cardiac catheterization performed at 7 years of age demonstrated no significant change. From 9 to 14 years of age, the systolic blood pressure gradient between the right arm and right leg was 20 to 25 mm Hg with normal upper extremity values. Echocardiography showed no aortic stenosis, aortic regurgitation, or left ventricular hypertrophy. Left ventricular contractility was normal. The Doppler gradient across the transverse arch ranged from 25 to 32 mm Hg.

When the patient was 14 years of age, a treadmill exercise tolerance test showed normal endurance (65 percent for age and gender), no ischemia, and no ectopy. One minute in the recovery period the blood pressure in the right arm was 202/50 mm Hg and in the right leg 99/53 mm Hg. By 9 minutes in the recovery period, the values were 118/60 and 93/50 mm Hg, respectively. After reviewing the data, conference participants agreed that the boy required no intervention at this point. Despite the presence of a large gradient during exercise, the lack of progression in the resting gradient over 12 years and the absence of hypertension at rest and left ventricular hypertrophy indicated that his gradient was not impairing cardiac function. The location of the coarctation made the risks associated with potential dilation, stenting, or surgery unacceptable.

An MRI was performed as a way of assessing left ventricular mass and obtaining a non-invasive image of the aorta. This study (Figure 1) showed gradual tapering of a leftward and superiorly oriented transverse aorta to a minimum diameter of 12x10 mm distal to the origin of the left carotid artery. The transverse arch then became dilated and tortuous where it joined the descending aorta. The left carotid and left subclavian arteries were approximately half the size of their right-sided counterparts. There were no stenoses in the descending aorta. The renal, celiac, and superior mesenteric arteries were normal. Left ventricular mass, volume, and contractility were normal. All of these factors supported the non-interventional plan.

Case 2: Intracardiac mass in an infant

A 10-day-old, full-term neonate was evaluated in our Lexington satellite clinic for a murmur detected at 36 hours of age. He was thriving without cardiac symptoms. On physical examination, there was a grade 2/6 early to mid-systolic murmur at the mid- to lower-left sternal border. Diastole was clear. An electrocardiogram was normal. Our physicians felt that the murmur probably represented a ventricular septal defect. Because of the child's young age and elevated pulmonary vascular resistance which could limit shunting, an echocardiogram was obtained to evaluate the size of the potential defect. The interventricular septum was intact; however, a large, solitary, 10x10 mm circular mass was seen in the left ventricular outflow tract, attached to the anterior mitral valve leaflet.

The infant was admitted to Children's and subsequent cerebral CT scan, renal ultrasound, skeletal survey, and ophthalmologic and neurological evaluations were performed. There were no abnormalities associated with tuberous sclerosis.

Because of concern for embolization or progressive obstruction, our staff decided to resect the mass. Via a trans-aortic approach, the tumor was found to be broadly attached to the anterior mitral valve leaflet. The tumor was resected, although a thin layer was left attached to the mitral valve to avoid surgical perforation of the thin leaflet. The postoperative course was uneventful. An echocardiogram performed two days after surgery demonstrated no residual left ventricular outlet obstruction, mild mitral regurgitation, and no mitral stenosis or aortic regurgitation.

Cardiac tumors are uncommon. In infants, this type of tumor is more likely to be a rhabdomyoma. It is usually multiple and intramyocardial, although single, intraluminal tumors can occur and can become smaller or resolve after birth. This tumor is frequently (30Ü80 percent of the time) associated with tuberous sclerosis.

The location and solitary nature of the tumor in the absence of signs of tuberous sclerosis increased the likelihood that this tumor represented another cell type. However, histology demonstrated typical features of a rhabdomyoma. This infant, therefore, had an atypical presentation of an uncommon condition.