John E. Capehart

Comparison of clinical and morphologic cardiac findings in patients having cardiac transplantation for ischemic cardiomyopathy, idiopathic dilated cardiomyopathy, and dilated hypertrophic cardiomyopathy.

This article compares intergroup and intragroup clinical and morphologic findings in patients with ischemic cardiomyopathy (IC), idiopathic dilated cardiomyopathy (IDC), and dilated hypertrophic cardiomyopathy (HC) undergoing cardiac transplantation (CT). Few previous publications have described findings in native hearts explanted at the time of CT. The explanted heart in 92 patients having CT was examined in uniform manner with particular attention to the sizes of the ventricular cavities and the presence of and extent of ventricular scarring. Of the 92 hearts examined, 47 had IC, 35 had IDC, and 10 had dilated HC. Although considerable degrees of intragroup variation occurred, the mean degree of left ventricular dilatation was similar among the patients with IC, IDC, and dilated HC. All patients with IC had left ventricular free wall scarring more extensive than that involving the ventricular septum, but the intragroup variation in the amounts of scarring was considerable. Nine of the 10 patients with dilated HC also had ventricular wall scarring, but it was more extensive in the ventricular septum than in the left ventricular free wall and involvement of the right ventricular wall also was present. Eight (23%) of the 35 IDC patients also had grossly visible ventricular scars but they were small and only 1 of the 8 had coronary narrowing and that was not in the distribution of the scarring. Narrowing of 1 or more epicardial coronary arteries >75% in cross-sectional area by plaque was present in all 47 IC patients, in 8 of the 35 IDC patients (7 had no ventricular scars), and in none of the 10 dilated HC patients. Coronary angiography was the major clinical tool allowing separation of the IC, IDC, and HC patients. Coronary angiography did not detect narrowing in any of the 8 patients with IDC who were found to have coronary narrowing on anatomic study. Thus, among patients with IC, IDC, and dilated HC having CT, distinctive anatomic features allow separation of patients with IC, IDC, and dilated HC, but within each group considerable variation in left ventricular cavity size and extent of ventricular scarring occurs.

Effectiveness of cardiac transplantation for primary (AL) cardiac amyloidosis.

A patient is described in whom severe cardiac dysfunction developed from amyloid deposited entirely in the walls and lumens of intramural coronary arteries, underwent cardiac transplantation, survived another 69 months, died suddenly, and at necropsy had amyloid again limited to the walls and lumens of the intramyocardial coronary arteries in the donor heart. The rather lengthy survival in this patient and the 118-month survival in a similar previously reported patient suggests that cardiac transplantation may be an appropriate procedure for some patients with cardiac amyloidosis.

Heart transplantation for undiagnosed cardiac sarcoidosis.

H transplantation (HT) for cardiac sarcoidosis is rare. Only 6 patients having HT for cardiac sarcoidosis have been previously reported (Table 1). In 2 of them, the diagnosis of cardiac sarcoidosis was not known at the time of HT. In this report, we describe a patient who underwent HT for presumed idiopathic dilated cardiomyopathy (IDC), but on examination, the explanted heart was found to have extensive cardiac sarcoidosis.

Morphologic features of cardiac sarcoidosis in native hearts of patients having cardiac transplantation.

Described herein are 10 patients who underwent cardiac transplantation (CT) for severe chronic systolic heart failure resulting from cardiac sarcoidosis. None had the diagnosis of sarcoidosis established before CT except for the 3 patients who earlier had had a portion of left ventricular wall excised for insertion of a left ventricular assist device and non-caseating granulomas were present in the removed myocardium. Although none of the 10 patients had significant narrowing of any of the epicardial coronary arteries, all had focal scarring of the walls of the left and right ventricles and ventricular septum and all had dilated ventricular cavities. The patients with the most ventricular wall scarring tended to have the fewest sarcoid granulomas in the ventricular walls. Two patients had no sarcoid granulomas in the excised heart although one did have typical sarcoid granulomas in the portion of left ventricular wall excised to insert a left ventricular assist device. Patients with cardiac sarcoidosis severe enough to warrant CT had characteristic cardiac ventricular morphologic findings, and no dysfunction of other non-cardiac organs, making clinical diagnosis of cardiac sarcoidosis rather difficult.

Morphologic features of the recipient heart in patients having cardiac transplantation and analysis of the congruence or incongruence between the clinical and morphologic diagnoses.

AbstractCardiac transplantation (CT) has been one of the great medical advances of the last nearly 50 years. We studied the explanted hearts of 314 patients having CT at Baylor University Medical Center Dallas from 1993 to 2012, and compared the morphologic diagnoses to the clinical diagnoses before CT. Among the 314 patients the morphologic and clinical diagnoses were congruent in 272 (87%) and incongruent in 42 (13%). Most of the incongruity occurred among the 166 patients with non-ischemic cardiomyopathy (non-IC) (36/166 [22%]), and of that group the major incongruity occurred among the patients with hypertrophic cardiomyopathy (7/17 [41%]), non-compaction left ventricular cardiomyopathy (NCLVC) (3/3 [100%]), mononuclear myocarditis (3/3 [100%]), arrhythmogenic right ventricular cardiomyopathy (ARVC) (4/4 [100%]), and cardiac sarcoidosis (8/8 [100%]). The phrase “non-IC” is a general term that includes several subsets of cardiac diseases and simply means “insignificant narrowing of 1 or more of the epicardial coronary arteries,” but it does not specify the specific cause of the heart failure leading to CT. A number of cardiac illustrations are provided to demonstrate the morphologic variability occurring among the patients with IC and non-IC.

Dramatically Different Phenotypic Expressions of Hypertrophic Cardiomyopathy in Male Cousins Undergoing Cardiac Transplantation With Identical Disease-Causing Gene Mutation

Described herein are certain findings in 2 male cousins who underwent cardiac transplantation for severe heart failure (HF), one of the diastolic type (ejection fraction ≈65%), and one of the systolic type (ejection fraction ≈20%), both the consequence of hypertrophic cardiomyopathy (HC), and each had identical disease-causing gene mutations. The implanted heart in one had normal-sized ventricular cavities and no grossly visible ventricular wall lesions (except in one papillary muscle) and the other patient had severely dilated ventricular cavities and multiple extensive ventricular wall scars. The heart mass and the age of onset of symptoms was similar in each patient. A number of other family members had evidence of HC.

Sildenafil—a possible treatment for acute pulmonary hypertension during cardiac surgery

Primary pulmonary hypertension has an annual incidence of 1 to 2 cases per million people in the general population and an onset in the third to fourth decades of life (1). It is a progressive disease that affects women twice as frequently as men; the mean survival time from diagnosis is 2.5 years (2). Patients with chronic congestive heart failure may develop secondary pulmonary hypertension, and this may become a limiting factor for acceptance into a cardiac transplantation program. The new cardiac graft will have a normal-sized right ventricle that may fail when exposed to the increased workload of a preexisting elevated pulmonary vascular resistance (PVR). It is vitally important to prevent acute elevations in PVR intraoperatively in patients undergoing cardiac transplantation with marginally acceptable levels of pulmonary hypertension. The mechanisms to treat an acute rise in pulmonary artery pressures in these circumstances are limited.

Cardiac Transplantation in Adults With Aortic Valve Disease With Focus on the Bicuspid Aortic Valve

The frequency of congenitally bicuspid aortic valves in patients having cardiac transplantation (CT) is unknown. We reviewed 243 explanted hearts in patients having CT at Baylor University Medical Center, Dallas from June 1997 through November 2011 to determine the frequency of a bicuspid aortic valve in this population. Of the 243 explanted hearts, 7 (2.9%) were found to have a congenitally bicuspid aortic valve: 3 had severe aortic valve stenosis and before CT had had the aortic valve replaced; the other 4 had normally functioning bicuspid valves and underwent CT for cardiomyopathy (ischemic in 1, idiopathic in 2, and hypertrophic in 1). Review of previously published reports of CT and aortic valve disease disclosed that 4 patients had had aortic valve replacement (AVR) from 2 to 8 years before CT, 3 had AVR or aortic valve repair of the donor heart at the time of CT, and 4 had AVR or transcatheter aortic valve implantation from 1 to 14 years after CT. Some of these aortic valve replacements, before, at the time of, or after CT were in patients with congenitally bicuspid aortic valves. In conclusion, congenitally bicuspid aortic valves were found in 7 of 243 explanted hearts in patients having CT at a single medical center in a 14-year period: 4 had functioned normally and 3 were severely stenotic. Previous reports of patients having AVR or repair before, during, and after CT were reviewed.

Management of pulmonary alveolar proteinosis by repeated bronchoalveolar lavage

Pulmonary alveolar proteinosis is a rare disease of the lung in which excessive thick granular phospholipoproteinaceous material accumulates within the air spaces. Many patients with this disease suffer from progressive dyspnea and cough that at times is accompanied by worsening hypoxia. At present, the only effective treatment is bronchoalveolar lavage, first described by Ramirez in 1963 (1). Presumably, this therapy is effective because it washes the proteinaceous debris from the airways and alveolar spaces. This case report describes the management of pulmonary alveolar proteinosis in 1 patient, who has undergone multiple, alternating, single-lung lavages over the past 10 years, with dramatic improvements in her symptoms following each therapy. We believe this to be the longest reported treatment of pulmonary alveolar proteinosis with repetitive bronchoalveolar lavage.

Chylopericardium following orthotopic lung transplantation

Chylopericardium is an uncommon condition, reported to occur following routine cardiac surgery, orthotopic heart transplantation, cardiac trauma, intrathoracic tumors, or infection. It has not, to date, been reported following uncomplicated orthotopic lung transplantation. This article describes chylopericardium following bilateral orthotopic lung transplantation.