E. W. Hancock

The syndrome associated with midsystolic click and late systolic murmur

Abstract A series of forty patients with midsystolic clicks, including twenty-eight patients with late systolic murmurs at the apex, is described. Evidence for the mitral valvular origin of the click and murmur in most cases is presented, in agreement with other studies published in recent years. Left ventricular cineangiograms in four cases showed mitral insufficiency in each instance. The incidence of associated features is emphasized, and it is proposed that these features constitute a distinctive syndrome. These features include a predominant female incidence, low or inverted T waves in leads II, III and aVF, prolonged Q-T interval, prominent U waves, atrial and ventricular extrasystoles, paroxysmal atrial and ventricular arrhythmias with occasional syncope or sudden death, familial occurrence, association with Marfans syndrome, Turners syndrome or atrial septal defect, and frequent neuropsychiatric disorders with hyperventilation syndrome. It is proposed that fibrosed or redundant mitral chordae tendineae due to various congenital or acquired causes, together with organic or biochemical dysfunction of the papillary muscles with repolarization delay, may link together the various features of this syndrome. Although generally benign, the prognosis occasionally may be more serious than has been considered previously.

Subacute effusive-constrictive pericarditis.

Clinical and hemodynamic observations are reported in 13 patients who demonstrated a distinct pathophysiologic form of compressive pericardial disease characterized by effusion into a free pericardial space associated with constriction of the heart by the visceral pericardium. The pericardial disease was idiopathic (nine patients) or subsequent to radiotherapy (four patients), with a maximal duration of symptoms of 16 months. Enlarged heart shadow, prominent paradoxical pulse, predominant systolic dip in the venous pressure pulse, and absence of atrial fibrillation were features that tended to distinguish the effusive-constrictive cases from those with noneffusive chronic constrictive pericarditis. The value of pressure measurements during combined cardiac catheterization and pericardiocentesis is emphasized in the differentiation of effusive-constrictive pericarditis from cardiac tamponade without constriction. Four patients were observed to progress through an effusive-constrictive phase to noneffusive constrictive pericarditis. Corticosteroids, pericardiocentesis, and other medical therapy produced some benefit but did not relieve constriction. The results of surgery were generally satisfactory.

Hypertrophic subaortic stenosis: Clinical and hemodynamic effects of long-term propranolol therapy☆

Abstract Thirteen patients with hypertrophic subaortic stenosis underwent right and transatrial septal left heart catheterization. Outflow obstruction was measured in the resting state and during a variety of stimulating maneuvers before and after the intravenous administration of 150 μg/kg of propranolol. The patients were then treated with orally administered propranolol and followed up for an average period of 17 months. Nine patients were available for a second catheterization, during which detailed hemodynamic measurements were repeated. Most patients had an initially favorable response to propranolol. However, 3 with an initially good response had a return of symptoms during the period of study. The initial clinical response appeared to correlate with the reduction of outflow obstruction seen after intravenous administration of propranolol. The long-term clinical course correlated with the hemodynamic findings at the second catheterization. The severity or lability of the outflow obstruction during the initial catheterization did not appear to have predictive value for the patients response to long-term therapy. Propranolol had a favorable effect on the symptomatic state of the patients, but did not appear to change the course of the underlying disease.

Left ventricular failure secondary to chronic pulmonary disease

Abstract Eight patients with chronic obstructive pulmonary disease and cor pulmonale in whom left ventricular failure developed are presented. None of them had a history or clinical evidence of systemic hypertension, coronary heart disease, valvular disease, alcoholism or other recognized causes of left ventricular dysfunction. Four of the patients had episodic left ventricular failure appearing during episodes of ventilatory failure, and four had left ventricular failure which was chronic and progressive. The appearance of left ventricular failure was suggested in several of the patients by change in character or severity of dyspnea, or by left ventricular heave or gallop rhythm. Roentgenograms revealed left ventricular enlargement in four cases, left atrial enlargement in four, pulmonary vascular congestion in seven and Kerleys B lines in four. Electrocardiograms demonstrated left ventricular hypertrophy or left ventricular conduction defects in three instances. The resting pulmonary artery wedge pressures were elevated in three patients, and left heart catheterization in two of them demonstrated similar elevation of left ventricular end-diastolic pressure. Autopsy in five patients showed biventricular dilatation and hypertrophy and pulmonary edema. Arterial hypoxemia, hypercapnia, high cardiac output and infection were examined as factors contributing to the left ventricular failure, but no one of them was uniformly prominent. It is concluded that left as well as right ventricular failure may develop secondary to chronic lung disease, probably due to multiple contributing factors.

Muscular Subaortic Stenosis Prevention of Outflow Obstruction with Propranolol

Eleven patients with muscular subaortic stenosis were studied by use of combined right and transatrial septal left heart catheterization. A variety of stimulating maneuvers were performed to induce or increase the functional outflow tract obstruction; 150 &mgr;g/kg of propranolol was administered intravenously, and the stimulating maneuvers were repeated. Outflow obstruction was latent (present only with stimulation) in four patients, labile (present intermittently at rest) in five patients, and persistent and severe in two patients. Propranolol abolished or significantly decreased the spontaneous variations in outflow obstruction occurring at rest, during the enhanced gradient in the post-exercise state, and during variations induced by isoproterenol infusion. The outflow obstruction induced by inhalation of amyl nitrite, the Valsalva maneuver, and premature ventricular contractions was only inconsistently prevented. Long-term oral propranolol therapy has been of significant symptomatic benefit in all the patients with latent or labile outflow obstruction and is considered the treatment of choice in these groups. In one patient with persistent outflow obstruction, an increase in symptoms occurred when oral propranolol was administered, and the drug was discontinued. Evidence is presented to support the concept of a cycle of obstruction and hypertrophy in the natural history of this disease. This vicious cycle might be prevented by propranolol.

Ventricular function in atrial septal defect

Abstract Ventricular function was evaluated in twenty-five adult patients with atrial septal defects, by combined right and left heart catheterization and hemodynamic measurements at rest and during exercise. Systemic cardiac output was measured by the dye dilution method and by the Fick method using superior and inferior vena caval samples to determine mixed venous blood oxygen content according to formulas determined in patients without intracardiac shunts. The resting systemic cardiac output was consistently below the age-related normal value but was associated with a left ventricular end-diastolic pressure (LVEDP) which for the group was significantly below normal. It is concluded that the decreased systemic cardiac output is due to impaired delivery of blood to the left ventricle secondary to the left to right shunt and does not represent an intrinsic abnormality of the left ventricle. Using exercise as a stress, three distinct patterns of ventricular function were found. In young asymptomatic patients, both ventricles functioned normally. In a somewhat older group, right ventricular failure was present but left ventricular function remained normal. In the oldest patients, both right and left ventricular failure were present. Left ventricular failure in the absence of right ventricular failure was not demonstrated in any of these patients. Clinical congestive heart failure was present in patients with evidence of right ventricular failure and normal left ventricular function on exercise, as well as in patients with biventricular failure. The data support the concept that the functional and structural impairment that occurs in response to overload of one ventricle is a generalized myocardial response with involvement of and eventual failure of both ventricles and that this is the usual mechanism for left ventricular failure in patients with atrial septal defects.

Mechanisms of Pulsus Alternans

The mechanisms of pulsus alternans were studied in three patients by cineangiographic determinations of left ventricular volume. In two patients with left ventricular disease, pulsus alternans occurred without detectable variation in left ventricular enddiastolic pressure (LVEDP) or end-diastolic volume (EDV), although in the second case these values did alternate in the initial postextrasystolic beats. Another patient with normal left ventricular function had brief postextrasystolic pulsus alternans associated with LVEDV alternations. Twenty-nine patients with valvular aortic stenosis who showed pulsus alternans during left heart catheterization were also studied. Persistent alternation in LVEDP occurred in eight, with transient LVEDP alternation appearing after extrasystoles in 22 cases. Cardiac cycle length and diastolic interval alternation occurred inconsistently.

measurement of systemic cardiac output at rest and exercise in patients with atrial septal defect

Abstract The problem of accurate measurement of systemic cardiac output at rest and during exercise in patients with atrial septal defect was studied by three interrelated approaches. 1. (1) The optimal method of approximation of mixed venous blood oxygen saturation from the oxygen saturation of superior and inferior vena caval samples was evaluated by comparisons with pulmonary arterial samples in patients without intracardiac shunts. Optimal formulas derived by regression analysis were as follows: Mixed venous blood at rest = (3 SVC + 1 IVC ) 4 Mixed venous blood during exercise = (1 SVC + 2 IVC ) 3 2. (2) Arterial dye-dilution curves after left ventricular injection were established as a reliable method for determination of cardiac output by comparison with standard Fick determinations in patients without intracardiac shunts. 3. (3) Fick cardiac output values using the derived formulas were compared with dye-dilution values following left ventricular injection in patients with atrial septal defect. A close correlation was noted. It is concluded that systemic cardiac output may be measured with reasonable accuracy both at rest and during exercise in patients with atrial septal defect by either the Fick or the dye-dilution method.

Coronary sinus rhythm in sinus venosus defect and persistent left superior vena cava

Abstract The frontal P wave axis was +15 ° or less (inverted P wave in lead III) in 8 of 10 patients with secundum atrial septal defect of the sinus venosus type or with left superior vena cava. A similar leftward P wave axis was present in 14 of 20 patients with left superior vena cava associated with other congenital heart lesions but was present in only 3 of 62 patients with uncomplicated atrial septal defect and in only 20 of 150 unselected children. The leftward P waves are thought to arise from an accessory pacemaker in the coronary sinus region of the A-V node, persisting from embryonic life when the left superior vena cava persists, or assuming pacemaker function when the S-A node is deficient in association with sinus venosus defect or is damaged by surgery. The association of sinus venosus defect with left superior vena cava is pointed out.

Aortic Valve Replacement With and Without Coronary Artery Bypass Surgery

Twenty-eight patients who underwent aortic valve replacement and coronary artery bypass grafts (group A) were compared, with respect to clinical presentation, operative mortality and morbidity and follow-up clinical status, with 40 patients who had normal coronary arteriograms and underwent aortic valve replacement only (group B). Groups A and B had a similar incidence of angina pectoris, cardiomegaly, and radiographic evidence of congestive heart failure prior to operation. Group A had a higher incidence of positive history of congestive heart failure, electrocardiographic changes indicative of an old myocardial infarction, and pure aortic regurgitation or mixed aortic stenosis-aortic regurgitation. In patients with pure aortic stenosis, the aortic valve area was greater and the aortic valve gradient was lower when there was associated coronary artery disease. The operative mortality was 14.3% in group A; 0% in group B. Postoperative morbidity was similar, except for a higher incidence of perioperative myocardial infarction in group A (10.7% vs 0%). There have been no late deaths in group A; four in group B. In the survivors, 23 of 24 group A and 36 of 36 group B patients are in NYHA class I or II, with good relief of symptoms. We conclude that the diagnosis of coronary artery disease in aortic valve disease is difficult to make without coronary arteriography. Combined aortic valve replacement and coronary artery bypass graft surgery carries a higher operative mortality than aortic valve replacement or coronary artery bypass graft surgery alone, but the clinical results at 1-3 year follow-up are equally satisfactory.