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Congenital apical left ventricular aneurysm is a rare clinical entity that is different from congenital left ventricular diverticulum. This aneurysm usually occurs as an isolated anomaly. Its clinical presentation varies, and it is usually diagnosed by exclusion.
Herein, we report the case of a 54-year-old man who experienced progressively increasing symptoms of congestive cardiac failure. Through the use of contrast echocardiography and angiocardiography, and upon histopathologic examination, he was diagnosed to have a congenital apical left ventricular aneurysm. He was successfully treated by means of left ventricular aneurysmectomy. We discuss the process of diagnosis and surgical correction of the aneurysm, and we briefly review the pertinent medical literature.
Congenital apical left ventricular (LV) aneurysm, a rare clinical entity that differs from congenital LV diverticulum, usually occurs as an isolated anomaly. The clinical presentation of congenital apical LV aneurysm varies, and the aneurysm is usually diagnosed by exclusion.1–4 Here, we report the case of a patient who presented with progressively increasing dyspnea. He was diagnosed to have a congenital apical LV aneurysm, for which he underwent aneurysmectomy. The extreme rarity of the disease entity, the use of angiocardiography and contrast echocardiography for diagnosis, the process of surgical correction, and a brief review of the medical literature form the basis of this report.
In January 2007, a 54-year-old man was referred to our institute with progressively increasing symptoms of dyspnea (New York Heart Association functional class IV) of 2 years' duration. Notable clinical findings included an intermittently irregular pulse, blood pressure of 124/80 mmHg, cardiomegaly, a systolic precordial thrill, a grade 3/6 systolic murmur that was loudest at the apical area, and hepatic enlargement. Chest radiography revealed cardiomegaly that chiefly involved the LV, and a prominent localized bulge with a curvilinear calcified rim at its apex. Electrocardiography showed atrial fibrillation, left-axis deviation, and left anterior hemiblock.
With use of a Hewlett-Packard Sonos 5500® echocardiography system with a 3.5-MHz transducer (Hewlett-Packard Company; Palo Alto, Calif), transthoracic 2-dimensional, color-flow, and Doppler echocardiography revealed LV and left atrial enlargement, normal cardiac valves, and an LV ejection fraction of 0.30. Cardiac catheterization that was performed for diagnostic confirmation of this unusual presentation revealed an elevated pulmonary capillary wedge pressure (35 mmHg) and elevated LV end-diastolic pressure (systolic/end-diastolic pressure range, 110/0–35 mmHg). Selective coronary arteriography showed normal coronary arterial origin, course, and distribution, without any disease (Fig. 1). Left ventriculography showed a 5 × 4-cm aneurysm in the apical region, with a filling defect within the cavity; the aneurysm was connected to the LV by an elongated neck. The aneurysmal sac had a curvilinear, calcified rim (Fig. 2). The patient was referred to surgery for aneurysmectomy.
Intraoperatively, the LV was found to be dilated and hypertrophied, and a large saccular aneurysm that measured 5 × 4 cm arose from the apex of the LV (Figs. 3A and and3B).3B). The anterior wall of the aneurysm was fibrotic, and the posterior wall was calcified and stiff, with normal endocardium and trabeculae. The aneurysmal cavity was full of thrombus. The mitral valve and chordae tendineae appeared normal (Figs. 3A and and3B).3B). The aneurysm was excised, and the defect of the LV wall was closed by use of interrupted 2-0 Prolene sutures over Teflon strips (Fig. 3C). Histopathologic examination of the resected specimen revealed fibrous tissue intertwined with muscle fibers.
The patient's postoperative course was uneventful. At 12 months of follow-up, he remained asymptomatic, and he was prescribed digoxin and diuretic agents. Echocardiography upon follow-up showed no residual defect, no mitral valve regurgitation, and good LV function (ejection fraction, 0.60).
Congenital ventricular aneurysms are localized protrusions of the free wall of the ventricles of the heart. Since the initial description of congenital apical LV aneurysm by Swyer and co-authors5 in 1950, there have been infrequent case reports of this malformation's location in the apex, in the annular subvalvular region of the free wall of the LV, and, rarely, in the muscular portion of the interventricular septum.1–9
No definite pathogenic explanation has been proposed regarding this anomaly. Theories include focal weakening of the ventricular wall caused by intrinsic abnormality during embryogenesis or by prenatal Kawasaki disease. Other possibilities include stenosis, hypoplasia, or localized intimal proliferation of the coronary arteries that causes myocardial infarction during infancy.5–10 In contrast with congenital LV diverticula, LV aneurysms are isolated congenital defects with a wide communication channel in the ventricular cavity, and with a wall (at times, calcified) that is composed of a single layer of fibroelastic tissue without the ventricular muscle layer and that shows paradoxical expansion during systole. Seventy percent of congenital LV diverticula are associated with congenital midline thoracoabdominal defects and congenital cardiac malformations.1–10
Most LV aneurysms are acquired and are secondary to myocardial infarction, hypertrophic cardiomyopathy with midventricular obstruction, tuberculosis, Chagas disease, injury, or surgery. Congenital LV aneurysm is diagnosed by exclusion.1–4
Patients with congenital apical LV aneurysm may be asymptomatic, or they may present with supraventricular and ventricular arrhythmias, heart failure, peripheral embolism, endocarditis, cardiac rupture, tamponade, or even sudden death.1–6
Patients with LV aneurysms may have a normal chest radiograph, or cardiomegaly or protrusion may be seen at the region of the aneurysm. The electrocardiographic abnormalities include LV hypertrophy with or without strain pattern, and nonspecific ST-segment elevation in the precordial leads. Two-dimensional and Doppler echocardiography are sensitive methods for detecting an aneurysm in the interventricular septum, LV wall, subvalvular area, or congenital LV diverticulum.1–10
Cardiac catheterization and angiocardiography remain the definitive means by which to exclude coronary artery disease, confirm the diagnosis, and define anatomic details. Of magnetic resonance imaging (MRI) techniques, phase-contrast cine sequences have been shown to define the cardiac morphology and function most accurately, leading in turn to the correct diagnosis of LV aneurysm.1–10 Despite isolated case reports of successful diagnosis through the use of MRI, the chief limitations are its availability and the need for highly specialized staff to interpret the data.
The case of our patient shows that the diagnosis of congenital LV aneurysm is facilitated by contrast echocardiography, angiocardiography, and intraoperative and histopathologic examination. The patient's normal coronary arteries, the exclusion of previous acute myocardial infarction, the existence of hypertrophic cardiomyopathy upon histopathologic examination of the resected specimen, and our exclusion of other possible causes in the clinical presentation enabled us to deduce that the LV aneurysm was most probably of congenital origin. This was further supported by evidence of calcification in the aneurysmal region, and by the presence of chordae tendineae extending from the LV wall and from the anterolateral and posteromedial papillary muscles.
Given the normal basic cardiac structure and myocardium in such patients, our interpretation is that the loss of kinetic energy via (assumed) paradoxical pulsation of the aneurysm is the cause of congestive heart failure. In our patient, the progressive dilation of the ventricular cavities, with the symptoms of congestive heart failure and the presence of thrombus within the aneurysmal sac, led to our choice of surgery in order to prevent future complications.
Address for reprints: Ujjwal K. Chowdhury, MCh, DNB, Department of Cardiothoracic & Vascular Surgery, All India Institute of Medical Sciences, New Delhi 110029, India