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Familial supravalvular aortic stenosis has been recognized as a distinct syndrome. A large family with five proven cases and a review of the literature on familial supravalvular aortic stenosis are presented. The diagnosis was substantiated in all 63 cases by cardiac catheterization, surgery, or postmortem examination.

Hemodynamic, respiratory, and pathological changes that occur during experimental fat embolism with free fatty acid have been evaluated in mongrel dogs. Most important were the elevation of pulmonary artery and pulmonary artery wedge pressure combined with sustained falls in the pulmonary pressures distal to the pulmonary capillary bed, indicating obstruction at the capillary bed. Systemic blood pressure fell. Decreasing dynamic pulmonary compliance, hypoxemia, and metabolic acidosis have also been consistent. These changes reflect the severe damage to the pulmonary capillary bed secondary to the fatty acid embolus. Results of pathological examination of the lungs revealed marked interstitial and alveolar edema. As the experiment progressed severe erythrocytic clumping and almost complete cessation of pulmonary capillary blood flow became obvious. The proposal that fatty acids are the underlying cause of the findings in tat embolization has been examined.

Two hundred fifty-five determinations of the protamine sulfate test, the staphylococcal clumping test, and the tanned red blood cell hemagglutination inhibition immunoassay were performed on 30 patients undergoing cardiovascular surgery. The level of serum fibrin-fibrinogen degradation products rose in all patients and reached the highest levels between the fifth and ninth postoperative day. After the first postoperative day, a fibrin-fibrinogen degradation product increase of greater than 10μg/ml within 24 to 48 hours was associated with clinically recognized thrombotic complications in only two out of five patients. Isolated levels of serum fibrin-fibrinogen degradation products as high as 67μg/ml could not be correlated with clinically evident thrombotic disease or serious complications of surgery. Increased excretion of urinary fibrin-fibrinogen degradation products was associated with, but not predictive of, acute ischemic renal failure.

Surgical resection has failed notably as definitive treatment for small cell carcinoma of the lung. Newer treatment programs combining intensive chemotherapy with radiation therapy achieve a significant response in about 85 percent of cases, with about 50 percent of patients showing clinically complete remission. Long-term survival without recurrence has been the outcome in a small minority of cases. A frequent mode of failure after treatment of limited disease is recurrence within the chest. The course of one patient treated early in this series suggests that exclusion of initial surgical resection from programs of combined treatment may be a serious omission. Since that time, four patients have undergone initial resection, apparently with uniformly favorable courses to date. Selection criteria based on staging factors are proposed. Admittedly, only a minority of patients will be suitable for this treatment at the time of first diagnosis. Much opportunity exists for improvement in survival rates of patients, even those with limited disease.

Supraventricular arrhythmias continue to complicate the postoperative course of patients undergoing myocardial revascularization. In a previous study, we showed a decrease in arrhythmias if patients were given digitalis prior to operation. Since that time we have made two changes—propranolol is no longer discontinued prior to operation and cold hyperkalemic cardioplegic solution is routinely used. To assess the affect of these changes on arrhythmias, we repeated the previous study. One hundred twenty patients all receiving preoperative and postoperative propranolol were randomized into a control group and a digitalis-treated group. The incidence of supraventricular arrhythmia postoperatively was 21.4% in the control group and 3.1% in the digitalis group (p < 0.005). Therefore, we continue to advise preoperative digitalization in patients requiring coronary artery bypass and continue to maintain beta blocker and digitalis therapy in the postoperative period.

The association of intraventricular conduction defects and aortic valvular disease is widely recognized. This study was undertaken to evaluate the effects on survival of left bundle conduction defects (LBCDs) as a consequence of aortic valve replacement. A total of 133 patients were followed between 1 and 70 months after operation, with a mean follow-up of 32.1 months. The incidence of intraoperative LBCDs was 31.6% or 42 patients. There were 13 deaths in the group of 42 patients with LBCDs compared to eight deaths in the group of 91 patients without such abnormalities (p < 0.01). Sudden death occurred in five of 42 patients with postoperative LBCDs and two of 91 patients with normal intraventricular conduction (p < 0.025). The survival rate in these patients with significant aortic stenosis and normal intraventricular conduction was 89.9%, whereas if LBCD had occurred after operation, the survival rate was 65.7% (p < 0.005). If the LBCD was accompanied by a left axis deviation, the survival rate was 21.7%. Sudden death may be due either to a tachyarrhythmia or perhaps to progression from LBCD to complete heart block or trifascicular block. It is important that this group of patients be monitored closely after operation. There may be an indication to insert prophylactic permanent pacemakers in this group.

The clinical, hemodynamic, and angiographic data on 92 patients with severe isolated aortic stenosis were reviewed to determine the incidence and mechanism of pulmonary hypertension. The status of each of these patients was determined 1 to 8 years after diagnosis by cardiac catheterization. Patients were divided into three groups on the basis of the pulmonary artery systolic pressure: group 1 (≤30 mm Hg), 46 patients; Group 2 (31 to 50 mm Hg), 31 patients; and Group 3 (>50 mm Hg), 15 patients. The prevalence of pulmonary hypertension was 50% (46/92) and that of severe pulmonary hypertension, 16% (15/92). There was no significant difference in age, aortic valve gradient, or valve area among the three groups. There was a significant positive correlation in left ventricular end-diastolic pressure (group 1, 15.5 ± 7.2 mm Hg; group 2, 23.3 ± 8.1 mm Hg; and group 3, 29.5 ± 5.8 mm Hg; R = 0.56, p < 0.01). There was also a significant negative correlation in left ventricular ejection fraction (group 1,67.5% ± 14%; group 2,62.3% ± 13.8%; and group 3 49.9% ± 18.3%; R = 0.43, p < 0.01). Of the 92 patients, 85 had aortic valve replacement with four (4.7%) hospital deaths. Follow-up showed excellent symptomatic relief in all three groups. Thirteen of the 15 patients in group 3, with severe pulmonary hypertension, had aortic valve replacement. There were no hospital deaths and only one noncardiac death at follow-up in Group 3 patients, and 11 of the 12 surviving patients were in New York Heart Association functional class I. We conclude that pulmonary hypertension is common in isolated aortic stenosis and is related to an elevated left ventricular end-diastolic pressure, frequently with preserved systolic function. Surgical results are excellent.

Between 1979 and 1986, 30 patients underwent replacement of the aortic valve and ascending aorta by a composite graft, with aortic wrapping of the graft. Thirteen patients had annuloaortic ectasia; six had DeBakey type I dissection (five acute, one chronic); three had DeBakey type II dissection (one acute, two chronic); three had left ventricular-aortic discontinuity caused by prosthetic valve endocarditis; three had sinus of Valsalva aneurysms after previous aortic valve procedures; and two had atherosclerotic aneurysms. Three patients died (10%). The mean duration of follow-up was 54 months. Fifteen patients consented to be restudied by intra-arterial digital subtraction angiography; studies were performed 6 to 58 months (mean 25 months) after composite graft replacement. Two patients had pseudoaneurysms at the right coronary anastomosis, which were repaired successfully. One patient showed persistent dissection beyond the distal aortic anastomosis; no reoperation has been done. One patient had pulmonary edema. Emergency study and reoperation showed disruption of the proximal aortic anastomosis and right coronary anastomosis. Anastomotic dehiscence after composite graft replacement is potentially lethal. Follow-up by means of intra-arterial digital subtraction angiography is simple and highly accurate. We suggest that dehiscences may occur early in the postoperative period and that restudy may be appropriate within a few months after operation.

Left ventricular function following aortic valve replacement has been evaluated in 15 consecutive patients. Cold potassium cardioplegia was utilized for myocardial preservation. Left ventricular function was assessed by radionuclide ventriculography performed preoperatively and 3 months postoperatively. The predominant lesion was aortic insufficiency in 10 patients and aortic stenosis in five patients. All patients demonstrated improved ejection fractions at 3 months. The mean increases of ejection fraction in the aortic insufficiency group were 20% from the anterior (Ant.) position and 12.5% from the left anterior oblique (LAO) position; in the aortic stenosis group they were 15.2% (Ant.) and 14.8% (LAO). It is our contention that cold potassium cardioplegia is an effective means of myocardial preservation and that it showed no measurable deleterious effect on left heart function in this group of patients.

The mechanisms of hypertension in coarctation remain to be clearly defined. In other hypertensive states, abnormal plasma renin activity (PRA) has been unmasked by the depletion of extracellular volume and the use of angiotensin antagonists. In a group of patients with coarctation, preoperative and postoperative evaluations of the renin-angiotensin system have been performed. Before operation, a group of patients with coarctation and a group of normal control subjects both underwent salt restriction followed by diuresis. A standard angiotensin antagonist (saralasin) test was performed on the patients with coarctation, and they demonstrated excessive renin-angiotensin activity compared to the control subjects. Following operation, paradoxical hypertension developed in all of the patients. Repeat saralasin test in these patients again revealed excessive angiotensin activity in the same patients as preoperatively. It appears that the renin-angiotensin system plays a more active role in coarctation than previously believed.

Excision of bullous cysts combined with pleural abrasion and tracheostomy appear to be the only surgical procedures available which afford significant improvement. The necessity of preoperative evaluation and meticulous preparation cannot be overemphasized.

THIS report deals with the treatment of pulmonary embolism by the application of the partially occlusive Mortez1 clip to the inferior vena cava in 62 patients over a five-year period. The effectiveness of the clip in controlling recurrent pulmonary emboli was evaluated. The patency of the inferior vena cava has been determined in 31 of 43 patients surviving six months or more, and the degree of venous insufficiency has been examined.The Moretz clip is but one of many nonligation techniques described which offer the theoretical advantage of inferior vena caval patency while protecting the patient against a fatal pulmonary embolus.1 There have been many reports dealing with the various methods of achieving this using a filter,2 plication with suture,3 or staples,4 or with a clip, with5 or without compartmentalization.1 It seemed apparent that more information was needed to evaluate the long-term caval

1. An understanding of the physics of sound is essential for a better comprehension of refined auscultation, tone analysis, and the use of these instruments. 2. The detection of variations of the third heart sound should prove a valuable aid in predicting mitral disease. 3. The variations of the outflow sound should prove a valuable aid in determining early aortic lesions with the type of accompanying intimal changes. 4. The character of chamber timbre as distinct from loudness heard as the first and second heart sounds denotes more often the condition of heart muscle, and must not be confounded with valvular disease. 5. The full significance of sound shadows is uncertain. Cardiac sound shadows appear normally in the right axilla and below the left clavicle. Their mode of production is quite clear. 6. Both the third heart sound and the outflow sound may be heard with the ordinary stethoscope.