Joseph M. Ryan

Interrelationships of Hyperinsulinism and Hypertriglyceridemia in Young Patients with Coronary Heart Disease

Fasting serum lipid levels, glucose tolerance, and immunoreactive insulin concentrations of 50 young patients with coronary heart disease (CHD) and 30 control subjects were evaluated to study the interrelationships of these metabolic factors. Abnormalities in one or more of these factors could be shown in 90% of the patients and 20% of the control subjects. Thirty-four of the 50 patients had elevated cholesterol or triglyceride levels, or both, 30 had abnormally elevated or delayed insulin responses after glucose, and 17 had abnormal glucose tolerance. A significant correlation existed between serum triglyceride and insulin concentrations. When insulin levels were reduced by phenformin, triglyceride concentrations fell toward normal.These findings indicate that carbohydrate, insulin, and lipid abnormalities are rather prevalent in patients with CHD. Excessive insulin secretion secondary to mild glucose intolerance probably induces hepatic synthesis of triglycerides and hypertriglyceridemia. Dietary alterations or pharmacological agents may help to control some of the metabolic abnormalities associated with premature CHD.

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.

Mitral valve prolapse syndrome: Analysis of 62 patients aged 60 years and older

Sixty-two patients diagnosed as having mitral valve prolapse, 60 to 81 years old, presented with disabling chest pain (20), symptoms of arrhythmias including palpitations and syncope (16), or mitral regurgitation (MR) with symptoms of congestive heart failure (26). The diagnosis of MVP was made on the basis of a combination of classic auscultatory, echocardiographic and angiographic findings. Thirteen of the 20 patients with chest pain had normal coronary angiograms and 7 had significant coronary artery disease (CAD). Patients with CAD could not be differentiated by clinical presentation alone. Furthermore, the incidence and types of arrhythmias, the presence of a positive stress test, and hemodynamic findings were similar in all patients in this group whether or not CAD was present. The 16 patients with palpitations had a broad spectrum of rhythm disorders, including both supraventricular and ventricular arrhythmias. Two patients had prehospital sudden death and 2 others had systemic emboli. Twenty-one of the 26 patients with MR had valve surgery. Intraoperatively the valves were described as enlarged, floppy and with redundant leaflets. Histologic examination showed extensive myxomatous changes throughout the valve leaflets. Thus, mitral valve prolapse is a cause of symptomatic heart disease in the elderly. It has a predictable pattern of clinical presentation and should be considered in the differential diagnosis of older patients with disabling chest pain and arrhythmias and as the cause of progressive or severe MR.

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.

Pulmonic Regurgitation Following Staphylococcal Endocarditis An Intracardiac Phonocardiographic Study

Isolated pulmonic regurgitation was found in a 30-year-old man 5 years after an episode of bacteriologically proved and successfully treated staphylococcal endocarditis. Intracardiac phonocardiography was useful in confirming the diagnosis, and simultaneous intracardiac pressure and sound recording afforded a plausible explanation for the unusual murmur of organic pulmonic insufficiency.

Experiences with Pulsus Alternans Ventricular Alternation and the Stage of Heart Failure

Three years experience with pulsus alternans, including its production with exercise, has shown that alternans may exhibit at least 3 types of behavior, and has led the authors to believe that the type of behavior a patient presents is related to the degree of heart failure present. Those patients who developed alternans following exercise were not in cardiac failure clinically, and yet hemodynamic data indicated myocardial insufficiency. A sustained pulsus alternans at rest seems to have no special prognostic significance in heart failure.

Thrombotic Calcific Mitral Stenosis: Morphology of the Calcific Mitral Valve

We compared the morphology of the calcific stenotic mitral valve (CSMV) with noncalcific stenotic mitral valves (NCSMV) removed at surgery; control valves were obtained at autopsy. X-rays of the excised valves permitted localization and quantitation of calcification. A classification of CSMV applicable to noninvasive techniques based on this methodology is presented. Moderate to heavily CSMV had greater weight, volume, specific gravity, weight per area, with smaller orifice size when compared with NCSMV and controls. Leaflet mobility was obliterated in moderate to heavily CSMV, while most NCSMV had some degree of leaflet mobility.Surface morphology was strikingly different in CSMV with 1) surface ulceration, due to eruption of the underlying calcific focus through valvular endothelium, 2) thrombosis in the areas of ulceration ( associated with symptomatic arterial embolization in four patients), 3) whisker formation, filamentous stalks along the line of valve closure.Calcification in the CSMV is viewed as an active, progressive process resulting in altered physical characteristics of the valve, progressive leaflet immobility and orifice narrowing, and eruptive surface changes with thrombus formation and arterial embolization arising from the CSMV itself. Clinical implications and a rationale for more precise classification of mitral stenosis on the basis of valvular calcification are presented.

Left Atrial and Left Ventricular Sound and Pressure in Mitral Stenosis

Left atrial and left ventricular pressure and sound were recorded at the time of mitral commissurotomy in 18 patients with severe, isolated, noncalcific mitral stenosis and sinus rhythm. Catheter tip micromanometers were used, and in eight studies, two equisensitive micromanometers were employed to examine pressure crossover relationships. Analysis of the left atrial and left ventricular sound and pressure crossover relationships is presented; viewed in the perspective provided by cineangiographic studies and surgical observations, a unifying concept of the auscultatory events in mitral stenosis is proposed.Beginning with left ventricular pressure rise, the left ventricle drives the mitral complex through its ascent (or eversion) phase toward the left atrium, clearing the anterior leaflet from the left ventricular outflow tract. The ascent (eversion) terminates abruptly at the first heart sound (S1). Following systole, left ventricular and left atrial pressure fall together after v peak, during and after pressure crossover, until the point where left atrial pressure fall ceases abruptly (opening snap notch), left atrial pressure exceeding that in the left ventricle, and the mitral opening snap occurs. During this interval, the mitral valve complex descends or inverts toward the left ventricle and terminates abruptly with the mitral opening snap. Thus, the mitral opening snap may be considered as the reciprocal of the delayed, accentuated S1, in noncalcific mitral stenosis in sinus rhythm.

The Influence of Advanced Congestive Heart Failure on Pulsus Alternans

Evidence is presented which indicates that in certain instances pulsus alternans may be abolished or diminished in the presence of advanced congestive heart failure. The observations suggest that in severe congestive failure the ventricular filling pressure may increase to the point where it causes the diastolic stretch of the left ventricle to be more equal from one beat to the next. This in turn would bring about more uniform systolic ejections and hence ventricular alternation would disappear or be lessened in degree.

Vascular Malformation Overlying the Pulmonary Artery Simulating a Patent Ductus Arteriosus

An arteriovenous fistula with aneurysmal dilatation involving branches of both coronary vessels and the pulmonary artery is discussed. The malformation was limited to the main pulmonary artery and presented a small left-to-right shunt without symptoms. Ligation of the supplying vessels resulted in the disappearance of a murmur typical of a patent ductus arteriosus.