Mary E. Fontana

The Varying Clinical Spectrum of the Systolic Click-Late Systolic Murmur Syndrome

Striking auscultatory variations with changes in posture were observed in 30 individuals with either mid-late systolic clicks, late systolic murmurs, or both, particularly in the upright position. Twenty-seven patients had late systolic murmurs; in nine, however, the murmur was not heard until assumption of the sitting position. Mid-late systolic clicks were heard in 20 patients while supine and in three only on sitting. Click movement (usually toward the first sound) was common during sitting or standing. Late systolic murmurs became holosystolic in 25 patients. In 20, the murmur did not become holosystolic until standing. Systolic whoops, not heard in the supine position, developed on assumption of the sitting position (three patients) or standing (three patients). With prompt squatting, the auscultatory findings reverted to those heard in the supine position in eight of nine patients. All observations were confirmed with phonocardiograms. Structural alterations in the mitral valve complex resulting in systolic prolapse of leaflets into the left atrium occur in these patients. Posture related changes in mitral valve function most likely explain the auscultatory phenomena.

Evidence for progression from mild to severe mitral regurgitation in mitral valve prolapse.

Little information is available concerning the progression of mild to severe mitral regurgitation (MR) in patients with mitral valve prolapse (MVP). This study reports 86 patients, average age 60 years, who presented with cardiac symptoms, precordial systolic murmur, severe MR and a high incidence of MVP on echocardiography (57 of 75 [75%] ) and left ventriculography (61 of 84 [73%] ). Seventy-five surgically excised mitral valves appeared grossly enlarged and floppy. Histologic studies showed extensive myxomatous changes throughout the leaflets and chordae. Eighty patients had had precordial murmurs first described at average age 34 years, but the average age at which symptoms of cardiac dysfunction appeared was 59. However, once symptoms developed, mitral valve surgery was required within 1 year in 67 of 76 patients who had undergone surgery. Atrial fibrillation, present in 48 of 86 patients (56%), or ruptured chordae tendineae, present in 39 of 76 patients (51%), may have contributed to this rapid progression and deterioration. Additionally, 13 patients had a remote history of documented infective endocarditis. Twenty-eight patients had at least 1 type of serial clinical evaluation that indicated progressive MR in all 28 patients on the basis of changing auscultatory findings (24 of 26), progressive radiographic cardiomegaly (24 of 25), echocardiographic left atrial enlargement (4.3 to 5 cm in 11 patients) and angiographically worsening MR (14 of 15). Twenty-four of these patients had evidence of MVP on at least 1 of their initial studies. Thus, mild MR due to MVP and myxomatous mitral valves is a progressive disease in some patients with MVP.

Vasodilators and Prostaglandin Inhibitors in Primary Pulmonary Hypertension

Ten women with primary pulmonary hypertension had resting hemodynamic measurements taken before and after the nonparenteral administration of various vasodilators and prostaglandin inhibitors. Only sublingual isoproterenol, alone or combined with sublingual isosorbide dinitrate, effected a substantial (greater than 20%) drop in pulmonary vascular resistance; this decrease was accompanied by little change in pulmonary artery pressure. Isosorbide dinitrate was the only drug that elicited any reduction in pulmonary artery pressure; pulmonary vascular resistance decreased modestly. The oral administration of diazoxide, hydralazine, phentolamine, and tolazoline elicited little change in pulmonary artery pressure or resistance. Except for tolazoline, all these agents significantly decreased systemic blood pressure and resistance. Prostaglandin inhibition by indomethacin (acute and chronic dosing) increased pulmonary and systemic vascular resistances and reduced cardiac output. Aspirin combined with dipyridamole elicited no changes. The vasodilators and prostaglandin inhibitors studied evoked little improvement in resting pulmonary hemodynamic abnormalities in primary pulmonary hypertension.

Postural changes in left ventricular and mitral valvular dynamics in the systolic click - late systolic murmur syndrome.

Marked changes in the auscultatory pattern with posture have been noted in patients with mid-systolic clicks and/or late systolic murmurs (MSC-LSM). MSC tend to move earlier in systole and LSM become longer and often louder when patients assume upright posture. Systolic prolapse of the mitral leaflets with mild regurgitation account for MSC-LSM; earlier and greater prolapse with more and prolonged regurgitation associated with a reduced left ventricular volume (LVV) in the upright position would explain the auscultatory changes. Twenty-two patients with MSC-LSM were studied supine and at 45 degrees head-up tilt, recording intracardiac pressures, cardiac outputs, systolic time intervals, and performing LV cineangiography. Systolic prolapse of one or both mitral leaflets was demonstrated in all patients. Left ventricular end-diastolic and end-systolic volumes both decreased significantly at 45 degrees in all sixteen technically satisfactory studies. Greater mitral prolapse was noted upright in 12 of 14 studies with enough sinus beats to judge. The amount of mitral regurgitation was mild in all, and changes in amount from supine to upright posture could not be discerned angiographically. THE FINDINGS, SUGGEST THAT THE AUSCULTATORY CHANGES OCCURRING WITH UPRIGHT POSTURE IN PATIENTS WITH MSC-LSM are related to greater prolapse of the mitral leaflets which is associated with a small LVV in the upright position.

Mitral valve prolapse and pregnancy

Mitral valve prolapse (MVP) is the most common congenital heart lesion, and the diagnosis is frequently made in young women of childbearing age. The management of this disorder during pregnancy has not been well studied. Our investigation reviews the outcomes of 42 pregnancies among 25 patients with MVP diagnosed before conception by the characteristic auscultatory and echocardiographic findings. All patients with no other cardiovascular disorder tolerated pregnancy well and developed no remarkable cardiac complications. Furthermore, the incidence of antepartum and intrapartum complications or signs of fetal distress was not greater when compared with pregnant patients with no known cardiac disorder (p greater than 0.05). Congestive heart failure occurred in one case in which premature labor with coexisting toxemia was treated with the combined intravenous administration of a beta adrenergic tocolytic drug, a glucocorticoid drug, and a large volume of fluids.

Mitral valve prolapse: cardiac arrest with long-term survival☆

Cardiac arrest has been reported in patients with mitral valve prolapse; however, clinical characteristics and survival information are limited since most of the cases reported include autopsy data. Nine patients (2 male, 7 female) with mitral valve prolapse were identified who had cardiac arrest; ventricular fibrillation was documented in 8 patients; resuscitation was unsuccessful in 2. Eight had a history of palpitations (months to 15 years duration) and ventricular arrhythmias, 3 had a history (5-15 years) of recurrent syncope, and 1 was totally asymptomatic. Cardiac catheterization-angiographic studies in 8 patients demonstrated normal coronary artery anatomy and mitral valve prolapse. All 9 patients had auscultatory and echocardiographic evidence of mitral valve prolapse. Seven survivors (6 still alive) were followed from 3 to 14 years after cardiac arrest. A subset of patients with mitral valve prolapse and cardiac arrest is described in whom past medical history is compatible with cardiac arrhythmias or syncope, and whose long-term prognosis appears better than patients with other causes of cardiac arrest.

Tricuspid stenosis. Atrial systolic murmur, tricuspid opening snap, and right atrial pressure pulse

Observations on the atrial systolic murmur, the tricuspid opening snap, and the right atrial pressure pulse of tricuspid stenosis are presented, based on catheter manometer intracardiac sound and pressure recordings in five patients with hemodynamically significant tricuspid stenosis. The manometer-recorded right atrial pressure pulse of tricuspid stenosis differed from the normal, with (1) elevation of right atrial pressure, (2) different morphologic features (tall, spiky A wave complete before C; small V wave with an interruption, the tricuspid opening snap notch at termination of the gradual Y descent; a diastolic plateau, the relatively flat diastolic segment of the right atrial pressure pulse following the tricuspid opening snap notch prior to the next A wave), and (3) the relative lack of right atrial pressure and right atrial pressure pulse response with normal respiration. The atrial systolic murmur, recorded in the right ventricular inflow tract, was complete by S1; the crescendo-decrescendo atrial systolic murmur configuration paralleled the right ventricular-right atrial diastolic pressure gradient at the time of the atrial A wave. The right atrial contraction-relaxation process, as reflected by the right atrial A wave ascent and descent, was complete at the onset of ventricular systole with P-R intervals of 170 to 200 msec. Thus, the timing and configuration of the atrial systolic murmur reflected the timing and completion of the right atrial contraction-relaxation process prior to the onset of right ventricular systole and the configuration of the tricuspid diastolic pressure gradient. The tricuspid opening snap was recorded in the right ventricular inflow tract and occurred at the time of a notch at the termination of the Y descent of the right atrial pressure pulse V wave, while right atrial pressure exceeded right ventricular pressure. The sound-pressure events were consistent with angiographic and echocardiographic studies, which showed doming or ballooning of the mobile, fused, stenotic tricuspid valve into the right ventricle during the Y descent of the right atrial pressure pulse. The tricuspid opening snap occurred at the time of the termination of the diastolic movement of the fused tricuspid unit into the right ventricle. These observations are presented within the framework of previous studies in order to trace the development of medical ideas about the pathophysiologic basis for the sound and pressure events of tricuspid stenosis.

Safety and efficacy of inferior vena caval occlusion to rapidly alter ventricular loading conditions in idiopathic dilated cardiomyopathy

To investigate the safety and efficacy of inferior vena caval (IVC) balloon occlusion for preload alteration in humans, 13 patients with dilated cardiomyopathy were studied before and during repeated (total of 78) IVC occlusions. Left and right ventricular (LV and RV) micromanometer pressures were simultaneously measured and M-mode and 2-D echocardiograms were recorded at end expiration. Complications were limited to abdominal discomfort in 2 patients. With IVC occlusion, RV collapse fluoroscopically shifted the heart toward midline and ventricular septal motion was frequently disordered. Significant (p = 0.001) changes occurred in RV and LV systolic peak pressures (from 19 +/- 6 to 12 +/- 5 mm Hg and from 129 +/- 34 to 109 +/- 25 mm Hg, respectively). LV and RV end-diastolic pressures also decreased significantly (from 18 +/- 7 to 6 +/- 6 mm Hg and from 5 +/- 3 to 2 +/- 2 mm Hg, respectively) (both p less than or equal to 0.0055). Similarly, LV end-diastolic diameter decreased 13% (from 61 +/- 11 to 53 +/- 12 mm, p = 0.0002). Mean heart rate did not change significantly (from 76 +/- 19 to 78 +/- 21 beats/min). Thus, IVC balloon occlusion provides a safe method of repeatedly altering loading conditions in humans. This approach allows for acquisition of important information regarding cardiac chamber dynamics while minimizing the effects of reflex mechanisms and avoiding use of pharmacologic agents.

Aneurysmectomy for recurrent ventricular tachyarrhythmias

Abstract The usual indications for aneurysmectomy have been congestive heart failure or thromboembolism. A review of the literature reveals only five previously reported cases of aneurysmectomy performed primarily because of recurrent, intractable, ventricular tachycardia and in the absence of congestive heart failure. An additional case is here presented.

Sail Sound in Ebstein's Anomaly of the Tricuspid Valve

Ebsteins anomaly represents an anatomic, pathologic, and physiologic spectrum. There have been few hemodynamic correlates for the observed auscultatory events. Multiple components of the first sound and “ejection’ sounds are frequently described.Cardiac catheterization, intracardiac sound-pressure studies (Telco), and cineangiograms were performed in three patients with Ebsteins anomaly who had a prominent early systolic sound.The right ventricular pressure pulse was abnormal in all; an initial delta-wave configuration, followed by a more rapid pressure rise, produced a prolonged rise to peak pressure. The right ventricular pressure pulse is not that of a conduction defect alone; rather it suggests that the altered pattern of ventricular contraction and abnormal leaflet placement are contributing factors.The early systolic sound was recorded in the atrialized right ventricle or right ventricle in all. It occurred just after the peak of the c wave in the atrialized right ventricle. In the right ventricle the sound occurred at the point where initial slow delta portion of right ventricular pressure pulse gave rise to rapid upstroke. The early systolic sound most likely occurs when the large, sail-like tricuspid valve reaches the limit of systolic excursion. The sound has been designated as the “sail sound,’ and may be the most specific auscultatory event in Ebsteins anomaly.