Aortic dissection: a rare complication of osteogenesis imperfecta.

Osteogenesis imperfecta (OI) is an inherited connective tissue disorder, a group that includes Ehlers-Danlos syndrome, Marfan's syndrome and pseudoxanthoma elasticum. OI is a heterogeneous disease of collagen I biosynthesis characterized by variable clinical phenotypes, including skeletal and cardiovascular manifestations. A 65-year-old man with OI who had extensive prior successful cardiac valve surgeries is described. He survived for 18 years after his initial valve surgery, but died of multiorgan failure and sepsis after repair of a spontaneous type A aortic dissection. This is the fourth reported case of aortic dissection secondary to OI and illustrates the extensive cardiovascular pathology associated with OI. Aggressive management of arterial dissection risk factors, such as systemic arterial hypertension, is advocated for patients with OI.

Long-term clinical results with the Ionescu-Shiley pericardial xenograft.

From 1977 to 1987, 829 Ionescu-Shiley pericardial valves (Shiley, Inc., Irvine, Calif.) were implanted in 766 patients at the University of Ottawa Heart Institute. There were 476 patients who had aortic valve replacement, 234 who had mitral valve replacement, and 44 who had double valve replacement. The standard-profile design was used in 508 patients and the low-profile design in 321 patients. Follow-up was obtained for 97% of patients, with calculation of event-free probabilities. At 10 years the overall probability of freedom from structural failure was 48% +/- 7% after aortic valve replacement, 44% +/- 15% after mitral valve replacement, and 79% +/- 11% after double valve replacement. Although at 5 years the probability of failure was statistically lower with the low-profile design, this favorability was lost by 6 years. Freedom from structural failure was only 47% +/- 7% for the standard-profile valve at 10 years. Thus the probability of freedom from reoperation was only 46% +/- 7% after aortic valve replacement, 39% +/- 6% after mitral valve replacement, and 65% +/- 20% after double valve replacement at 10 years. Thromboembolism occurred in 69 patients, for a predicted freedom from this complication at 10 years of 79% +/- 3% after aortic, 73% +/- 7% after mitral, and 96% +/- 4% after double valve replacement. There were 31 cases of endocarditis. The 10-year predicted freedom from endocarditis, therefore, was 86% +/- 3% after aortic, 98% +/- 1% after mitral, and 97% +/- 1% after double valve replacement. A total of 221 operative and late deaths were recorded in this series. Prosthetic valve failure accounted for 27% of late deaths. The 10-year survival rates were estimated to be 56% +/- 5% (aortic valve replacement), 54% +/- 6% (mitral valve replacement), and 51% +/- 8% (double valve replacement). We concluded that the Ionescu-Shiley pericardial xenograft provides less than optimal clinical performance and its use has been discontinued.

Atypical hepatic vein leiomyoma extending into the right atrium: an unusual cause of the Budd-Chiari syndrome.

A 14-year-old male presented with a one week history of weakness, lightheadedness and vomiting. Bilateral pleural effusions were evident on chest radiography; electrocardiogram revealed decreased voltages. Echocardiogram, abdominal ultrasound and magnetic resonance imaging revealed a mass in an hepatic vein and the inferior vena cava extending up to and filling the right atrium. Under deep hypothermia and extracorporeal circulation the mass was removed en bloc. It originated from the hepatic vein. Pathology revealed a smooth muscle tumour intermediate between benign and malignant (atypical leiomyoma). This is the first reported pediatric primary leiomyoma of the hepatic vein. It caused the Budd-Chiari syndrome, a rare pediatric entity.

Do symptoms reflect a change in left ventricular function after aortocoronary bypass grafting in patients with depressed left ventricular function?

Seventy-nine patients with moderate to severe left ventricular dysfunction who underwent aortocoronary bypass grafting between 1971 and 1977 had follow-up heart catheterization at a mean interval of 3 years. Thirty-three patients (42%) had angiographic improvement in left ventricular function at follow-up and 18 (25%) had a decrease in left ventricular end-diastolic pressure. Fifty-eight patients (73%) had improvement in angina of at least one New York Heart Association class at follow-up. There was no correlation between late improvement in left ventricular function and improvement in angina. Improvement in left ventricular function did not correlate with preoperative indices of severity of coronary disease or with indices of completeness of surgical repair.

Submammary skin incision as a cosmetic approach to median sternotomy.

Median sternotomy is the incision of choice to allow access to the anterior mediastinum, heart, or both lungs. The vertical skin incision leaves an unsightly scar for many female patients. A bilateral submammary horizontal skin incision with dissection of a flap including the subcutaneous tissue and breasts allows exposure of the sternum so that a median sternotomy can be performed. Since November 1981, we have used this incision 40 times in female patients undergoing open heart surgery. The exposure of the mediastinum was excellent, and there were no difficulties in cannulating the ascending aorta for cardiopulmonary bypass. Complications associated with this incision are insignificant if close attention is paid to details.

Mitral valve replacement with Hancock porcine bioprostheses: up to 7-year follow-up.

From February 1976 to May 1981 at the University of Ottawa Heart Institute, 137 mitral valves were replaced with the Hancock porcine bioprosthesis. This study concerns 111 patients (55 men, 56 women) who had single-valve replacement. The average age of the patients was 53.0 years. The predominant lesion in the 102 patients who were receiving their first substitute valve was stenosis in 46% and regurgitation in 42%. Preoperatively, 82% were New York Heart Association (NYHA) class III or IV. Additional procedures were performed in 44 patients (39.6%). The 26 patients who had multiple valves replaced were considered only for durability studies. Hospital mortality was 8.9% for isolated replacement and 22% for replacement associated with aortocoronary bypass grafting. At the time of discharge, 50% of patients were in atrial fibrillation, 14% were taking warfarin and 82% antiplatelet agents. Follow-up was available for a total of 374 patient-years (average of 5.4 years per patient). The mean symptomatic improvement was 1.6 NYHA classes per patient. Actuarial analysis indicated a survival rate of 70% at 8 years. Of the 18 late deaths, 4 (22%) were valve-related. The rates of endocarditis and thromboembolism were 1.1% and 5.8% patient-year respectively. In the 137 prostheses at risk, intrinsic failure occurred at the rate of 4.7% patient-year for a cumulative rate of 35% at 8 years. The authors conclude that the Hancock porcine bioprosthesis provides good relief of symptoms in the mitral position with an acceptable rate of thromboembolism even without anticoagulation.(ABSTRACT TRUNCATED AT 250 WORDS)

Ionescu-Shiley pericardial xenografts: follow-up of up to 6 years.

The results of valve replacement with the Ionescu-Shiley pericardial xenograft compare favorably with results obtained with other bioprostheses. From March, 1977, to July, 1983, 497 Ionescu-Shiley pericardial valves were implanted in 463 patients at the University of Ottawa Heart Institute. There were 292 patients who had aortic valve replacement (AVR), 140 with mitral valve replacement (MVR), 28 with double valve replacement, and 3 with triple valve replacement. The survivors were followed regularly. Actuarial analysis of late results indicates an expected survival of 71% at 6 years for patients who underwent AVR and 72% at 3 years for patients who had MVR. The only valve-related deaths were due to endocarditis, which occurred at a rate of 3.9% per patient-year for aortic valves and 0.6% per patient-year for mitral valves. Despite a low usage of formal anticoagulation, embolic complications occurred at a rate of 1.4% per patient-year for aortic valves and 4.0% per patient-year for mitral valves. Five valves were removed for intrinsic failure after 36 to 72 months of follow-up. New York Heart Association Functional Class improved an average of 1.28 classes per patient.

Triple-vessel coronary artery disease: Effect of revascularization on late survival.

Of the first 600 patients to receive aortocoronary bypass grafts 383 had three diseased vessels, and of them 36 (9%) have died: 20 (5%) in hospital and 16 (4%) late. In 10 of those who died, ventricular function was normal, class I or class II and in the other 26 function was class III or class IV. For all ventricular classes except class IV, improvement in myocardial blood flow with operation was considerably lower than average in those who died. The leading cause of late death was congestive heart failure followed by myocardial infarction, and then by other cardiac complications. Of patients with good (normal, classes I or II) ventricular function, 3.5% died during a mean follow-up period of 27 months; and since, according to published reports, 1% of patients with triple-vessel disease without surgical intervention die each month, the data from this study suggest that survival in this group is improved by 23.5%. This study also shows that in patients with triple-vessel disease and good ventricular function, adequate coronary artery bypass operations (i.e., placement of three grafts or more) improves survival.