Orthotopic Heart Transplantation for Ankylosing Spondylitis Masquerading as Nonischemic Cardiomyopathy.

Described herein is a 48-year-old man who underwent orthotopic heart transplantation because of severe heart failure considered clinically due to idiopathic dilated cardiomyopathy, but examination of the operatively excised native heart disclosed classic features of ankylosing spondylitis. Orthotopic heart transplantation for this condition has not been reported previously.

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

Cardiac 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.

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.

Examination of isolated ventricular noncompaction (hypertrabeculation) as a distinct entity in adults.

Three patients (2 women) 36, 45, and 49 years of age underwent cardiac transplantation for what was diagnosed clinically as nonischemic dilated cardiomyopathy. Examination of the transthoracic echocardiogram and explanted heart in each disclosed marked hypertrabeculation involving the free wall of the very dilated left ventricle, a finding consistent with what has been termed "isolated ventricular noncompaction" (IVNC). Although these 3 cases anatomically fulfilled the echocardiographic definition of IVNC, review of previous publications containing gross photographs of the heart suggests that IVNC is overdiagnosed at least morphologically.

Some previously neglected examples of arrhythmogenic right ventricular dysplasia/cardiomyopathy and frequency of its various reported manifestations.

Four patients are described with either parchment-like thinning or partial but extensive myocyte depletion with severe fatty or fibrofatty infiltration of the free wall of the right ventricle in its outflow tract, including 2 previously reported patients who also had focal parchment-like thinning of the left ventricular free wall. Three had documented ventricular tachycardia, and the remaining patient had sudden death as his first and only manifestation of heart disease. Three patients had severe heart failure: in 1, it was fatal, and the other 2 underwent cardiac transplantation. Necropsy cases of parchment-heart syndrome before 1980 are reviewed, as well as large series of cases with arrhythmogenic right ventricular dysplasia (ARVD) reported subsequently. It is suggested that ARVD is not an ideal name for this condition, because malignant ventricular arrhythmias are not universal, the left ventricular free wall and/or ventricular septum are sometimes involved, and the name "ARVD" neglects the fact that severe heart failure may be prominent in these patients. The right ventricular wall can be thin or parchment-like, or it may not be thinned but consist mainly of adipose tissue with or without focal fibrous tissue and a few islands of myocytes. Nevertheless, because the name "ARVD" has been commonly used and recognized for >30 years, it is probably best retained for this condition.

Cardiac transplantation for cardiac sarcoidosis with initial diagnosis by examination of the left ventricular apical "core" excised for insertion of a left ventricular assist device for severe chronic heart failure.

Three quinquagenarians who underwent insertion of a left ventricular assist device (LVAD) because of severe heart failure and for whom histologic examination of the left ventricular apical "core" (removed to insert the device) showed noncaseating giant cell granulomas typical of sarcoidosis are described. Later, cardiac transplantation showed widespread sarcoid granulomas in the walls of the right and left ventricles and ventricular septum in 2 patients and extensive scarring in the third patient in the absence of coronary narrowing. Previously, 11 patients who underwent cardiac transplantation because of cardiac sarcoidosis had been reported, and in 1 of these patients, diagnosis was also initially made by examination of the left ventricular core excised at the time of insertion of an LVAD. In conclusion, excision of a portion of left ventricular wall to enable insertion of a therapeutic device (LVAD) can also serve as the means of definitive diagnosis of the underlying cardiac condition.