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A 2-year-old girl presented with signs and symptoms of cardiac tamponade preceded by upper respiratory tract infection. A two-dimensional echocardiogram was suggestive of an intrapericardiac tumor. Exploration of the pericardium revealed a large amount of dense amber-colored "coagulated material." Microscopic examination of the material and the pericardium was diagnostic of acute pericarditis. Based on this experience we propose the term pseudotumor of the pericardium for this form of coagulated pus. We also propose that this term should be added to the differential diagnosis of intrapericardiac tumor.

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BACKGROUND: We tested the hypothesis that plasma cholesterol lowering action of partial ileal bypass (PIB) is beneficial in mitigating accelerated transplantation coronary vasculopathy. METHODS: Forty-one New Zealand white rabbits were randomized to receive a normal (n = 21) or 1% cholesterol diet (n = 20). They underwent heterotopic heart transplantation with sham-PIB (n = 19) or PIB (n = 22) and immunosuppression with cyclosporine A (CyA). RESULTS: CyA increased plasma cholesterol of rabbits receiving a normal diet. This effect was mitigated by PIB (101 +/- 50 mg/dl CyA vs baseline 24 +/- 8, p < 0.001; vs 54 +/- 25 mg/dl with PIB, p < 0.05). In cholesterol-fed rabbits, PIB decreased plasma cholesterol levels (520 +/- 236 mg/dl PIB vs baseline 720 +/- 359, p < 0.05; vs 1502 +/- 253 mg/dl with sham PIB, p < 0.00001). Coronary arteries (CA) of 21 5-week survivors were evaluated by light microscopy and digital morphometry. No rejection was noted. Histologic study revealed vasculopathy in 3% of 705 native and 18% of 654 transplant CA (p < 0.05). Graft vasculopathy (GV) was present in 25% of 365 CA of sham-PIB and 10% of 289 CA of PIB rabbits (p = 0.07). In cholesterol-fed rabbits, GV was characterized by fatty proliferative lesions in 75% of 91 pathologic CA of sham and 21% of 28 pathologic CA of PIB rabbits (p < 0.05). Graft intimal hyperplasia was not correlated with cholesterol intake or PIB and was present in 18 of 119 pathologic CA. CONCLUSIONS: GV was characterized by fatty intimal proliferation, fibrous intimal hyperplasia, and a "mixed type." Fibrous intimal hyperplasia developed in native and transplanted hearts, and CyA seemed to promote this state. Hypercholesterolemia promoted fatty proliferative lesions, worsening GV. PIB significantly decreased total cholesterol and retarded fatty proliferation of CA of native and transplanted hearts but did not prevent intimal hyperplastic vasculopathy. Therapy of hypercholesterolemia is recommended to at least mitigate the fatty intimal proliferation of GV.

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Mortality is high for children with pulmonary atresia, intact ventricular septum, and important connections between the right ventricle and the coronary arteries because of myocardial ischemia: in systole, suprasystemic right ventricular pressure delivers deoxygenated blood to the coronary artery (or arteries) and in diastole, the right ventricle provides a lower resistance alternative to coronary perfusion of the myocardium. Tricuspid valve closure was performed in 10 such children. None had stenosis of native coronary arteries. A trial of tricuspid valve closure (by balloon) was performed in the cardiac catheterization laboratory in 5 of 10 patients. Seven of 10 children survived surgical closure of the tricuspid valve plus concurrent procedures; none had heart block. Two of the 3 nonsurvivors were probably in inoperable condition due to preoperative myocardial ischemia. Before operation, 4 patients had ischemic changes on electrocardiograms; these changes were abolished after operation. Three of 10 patients have had a Fontan operation with 2 survivors. We conclude that children with pulmonary atresia, intact ventricular septum, important connections between the right ventricle and the coronary arteries, and normal native coronary arteries should have surgical closure of the tricuspid valve within the first year of life and treated thereafter as patients with "tricuspid atresia."

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The submitral apparatus may play a predominant role in rheumatic mitral stenosis and should be evaluated aggressively with Doppler echocardiography, especially in patients in whom percutaneous mitral balloon valvotomy is being considered. The following case presents a patient with rheumatic mitral valve stenosis in whom some noninvasive clues alerted us to the presence of predominant submitral stenosis.

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Infections occurred in 52 of 400 patients (13%) undergoing coronary artery bypass operations from January 1987 to December 1990. The hospital courses of 5 patients (1.3%) in whom occult infections of the paranasal sinuses developed were reviewed. Only 1 patient had specific clinical findings of acute sinusitis (purulent nasal discharge). Computed tomography showed wall thickening, opacification, or air-fluid levels in one or more paranasal sinuses in each patient. All patients were successfully treated with surgical drainage and antibiotics. Risk factors for development of postoperative acute sinusitis include: prolonged tracheal intubation, airway colonization with nosocomial bacteria, inability to clear nasal secretions, sinus ostial obstruction, and critical organ system dysfunction. Physical examination and roentgenographic evaluation of the paranasal sinuses should be considered when postoperative sepsis of obscure etiology occurs.

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The four mechanical valve prostheses currently available on the U.S. market have evolved from a field of more than 50 valves produced for human implantation since the early 1950s (Tables 11-1 to 11-4). This literature establishes that good results can be achieved with a number of cardiac prostheses if properly used and monitored after implant. The current generation of valves have demonstrated ease of implantation, improved durability, good hemodynamic performance, and reduced thromboembolism and thrombosis with proper anticoagulation. The cost and complexity of completing PMA by the FDA, concern over product liability, and patent rights on design and raw materials have narrowed the choice of devices for surgeons in the United States and slowed the pace of new market entries. The evolution of mechanical valves has been reviewed and modes of valve failure reviewed when pertinent. Clinical expectations for earlier generation devices and present valves also are reviewed. Prostheses under evaluation are discussed along with considerations for valve implantation, surveillance, and anticoagulation. We have employed the SJM valve since about 1985. The proven good hemodynamic performance in small sizes and low profile have made its application well suited to the pediatric population and for smaller aortic roots. The well-guarded hinge mechanism and low probability for disc entrapment have facilitated its use in chordal sparing mitral replacements in our experience. Application in the tricuspid position also has been successful but requires close attention to anticoagulation.

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The case of a 68-year-old man with advanced symptomatic coronary artery disease and large aneurysm of the diverticulum of the ductus arteriosus is presented. Both the coronary artery disease and the aneurysm were successfully repaired at the same time via median sternotomy using cardiopulmonary bypass and a brief period of profound hypothermic circulatory arrest. To our knowledge, this is the 27th reported case of such an aneurysm in an adult.

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A total of 101 reparative and 389 valve replacement operations, isolated or combined with tricuspid annuloplasty or operations for coronary artery disease, were done for mitral incompetence (1975 to July 1, 1983). The patients undergoing repair as a group were younger and had less hemodynamic and functional derangement than those undergoing replacement. The prevalence of repair was less (p less than 0.001) for two surgeons than for the other four, even when possible differences in patient populations were taken into account by multivariate analysis. Five-year survival rate, including hospital deaths, was 76% after valve repair and 56% after valve replacement (p = 0.005). However, by multivariate analysis, valve replacement rather than repair was only possibly (p = 0.14) a risk factor. (Multivariate analysis in all patients undergoing mitral valve repair in the period 1967 to 1985 [n = 210] did not find the type of annuloplasty to be a risk factor.) The incidence of reoperation was no different after repair or replacement and there was no increase in the risk of reoperation late after repair. Endocarditis early or late after operation occurred in 11 of the 389 patients undergoing mitral replacement and in none of those undergoing repair (p = 0.08). The functional status of the patients was not different between the two groups. These data, and the experience of others, indicate the advantages of repairing rather than replacing the incompetent mitral valve whenever possible.

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Classical tetralogy of Fallot was repaired by a transatrial-transpulmonary approach in 61 of 70 patients treated surgically on one surgical service between July 1981 and December 1985. Their ages ranged from 2.5 months to 57 years (mean 52.9 +/- 104.2 months). The ventricular septal defect was closed through the right atrium and tricuspid valve in 60 patients and via the pulmonary artery in one patient with a subpulmonary defect. A pulmonary arteriotomy was made in 38 patients to expose the stenotic pulmonary valve for valvotomy and to begin the infundibular mobilization and resection. It was extended through a small pulmonary anulus primarily in 15 patients and secondarily in one, a minimal distance upon the right ventricular infundibulum to enlarge the outflow tract to 2 standard deviations larger than the mean normal pulmonary valve anulus diameter. There were no hospital or late deaths or reoperations among the entire group. The mean postrepair peak right ventricular/left ventricular pressure measured in the operating room was 0.52 +/- 0.165 for the entire group of 61 patients which indicates similar relief of pulmonary stenosis to that obtained by the classical transventricular repair. At follow-up between 2.3 and 53.8 months (mean 22.6) each patient was in New York Heart Association Class I and without cardiac medication. Postoperative cardiac catheterization studies performed in nine patients between 4 and 28 months after operation showed no residual ventricular septal defects and a mean peak right/left ventricular pressure of 0.42 +/- 0.13. This series indicates that successful repair of tetralogy of Fallot can be accomplished in most patients by a transatrial-transpulmonary approach, which may provide improved right ventricular function over the long term.

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This chapter is not meant to be an exhaustive review; it is meant only to highlight the clinical developments over the past decade in selected areas. Much remains to be learned about the management of many congenital cardiac defects, and only through further experience, curiosity, and imagination, coupled with careful and complete analysis of our activities, will we further refine and improve surgical management programs that are so critical to the patients we treat.

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Connection of an anomalous left superior vena cava to the left atrium is an uncommon lesion that is usually associated with other complex intracardiac malformations. A technique for diverting the anomalous caval return along the left atrial roof and into the right atrium is presented. This simple tunnel method avoids potential obstruction to systemic and pulmonary venous return and leaves viable atrial tissue comprising the majority of the new pathway. The technique and results in 7 patients are described.

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Thirty-one patients were operated on for benign thyromegaly extending to the thorax in an 11-year period at the University of Alabama in Birmingham. Neck mass (65%), dysphagia (36%), and dyspnea (32%) were the most common symptoms. All patients were euthyroid. Five patients had previous thyroid surgery. A thyroid scan was performed on 24-patients. Fourteen (58%) suggested a thoracic extension while ten (42%) failed to identify a thoracic extension. The indications for resection were increasing symptoms, increasing size despite the use of dessicated thyroid therapy, and to establish a diagnosis. The left thyroid lobe extended into the thorax more frequently (70%) than the right. Most patients had multinodular goiter (94%). Three patients had occult carcinoma (10%) and two patients had Hashimoto's disease. Median sternotomy combined with a collar incision to provide exposure for excision of intrathoracic thyroid extension was used in six patients. There was no operative mortality. There was no increase in operative morbidity and a slight increase in average stay from 5.3 days with a collar incision alone to 6.8 days with the combined incisions. Median sternotomy does not increase morbidity or mortality. Specific indications for more liberal use of sternotomy extension of a collar incision are proposed for the management of substernal and intrathoracic goiters.

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