Ann Thorac Surg 2000;69:1253-1255
© 2000 The Society of Thoracic Surgeons
CASE REPORTS
Successful myocardial volume reduction in a 9-month-old infant
Luca A. Vricella, MDa,
Steven R. Gundry, MDa,
Ranae L. Larsen, MDa,
Leonard L. Bailey, MDa
a Division of Cardiothoracic Surgery, Loma Linda University Medical Center and Children’s Hospital, Loma Linda, California, USA
Address reprint requests to Dr Gundry, Division of Cardiothoracic Surgery, Loma Linda University Medical Center, 11234 Anderson Ave, Loma Linda, CA 92354
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Abstract
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Left ventricular reduction has shown promise as a treatment for end-stage dilated cardiomyopathy, with restoration of the physiologic ratio between myocardial mass and left ventricular diameter. We present a case of successful partial left ventriculectomy utilizing both lateral and septal wall excision as treatment of dilated cardiomyopathy in a 9-month-old patient.
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Introduction
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Left ventricular reduction has shown promise as a treatment for end-stage dilated cardiomyopathy, with restoration of the physiologic ratio between myocardial mass and left ventricular diameter. We present a case of successful partial left ventriculectomy utilizing both lateral and septal wall excision as treatment of dilated cardiomyopathy in an infant.
A 9-month-old, 7.6-kg female developed progressive dilated cardiomyopathy diagnosed at 6 months of age, requiring hospitalization for congestive heart failure and transcatheter coil embolization of patent ductus arteriosus. Etiologic workup of cardiomyopathy was unremarkable. Physical examination was significant for a 2/6 systolic murmur over the cardiac apex and marked hepatomegaly. Chest roentgenograms demonstrated cardiomegaly and mild pulmonary edema; electrocardiogram was significant for biventricular hypertrophy. Endomycardial biopsy did not show histologic evidence of myocarditis. The therapeutic regimen included digitalis, furosemide, and captopril. Preoperative transthoracic echocardiogram (without any inotropic support) showed ejection fraction of 25%, fractional shortening of 9%, and left ventricular systolic and diastolic diameter of 4.3 and 3.7 cm, respectively. Moderate mitral valve regurgitation was noted. Because of progressive clinical deterioration, surgical options were reviewed, and the patient’s family declined cardiac transplantation.
On December 2, 1996, the patient underwent partial ventriculectomy with DeVega mitral annuloplasty and Alfieri mitral valvuloplasty. The left ventriculectomy was performed in the posterolateral wall of the left ventricle, between the anterior and posterior papillary muscles; this was carried to the mitral valve annulus, and extended to the apex and well into the interventricular septum (Fig 1). Twelve grams of myocardium (4.5 x 4.0 cm) were excised, and closure of the ventriculectomy and the apical septum were both accomplished primarily with a continuous 3-0 vicryl suture. Pericardial pledgets were used in the closure of the posterolateral wall of the left ventricle. The patient was easily weaned from cardiopulmonary bypass on low-dose dopamine and isoproterenol. The postoperative course was complicated by an apical ventricular septal defect secondary to dehiscence of the septal suture line, requiring reoperation with primary closure on postoperative day 15. Shortly after the procedure, the patient manifested mental obtundation. The patient had developed marked systolic hypertension, which necessitated aggressive antihypertensive therapy and was ultimately controlled with calcium antagonists. Computerized tomogram of the head revealed no evidence of focal lesions. With control of the high-output state, mental obtundation slowly resolved. The patient was eventually discharged on postoperative day 47, with no evidence of neurologic sequelae at 1-month follow-up. Predismissal echocardiography showed an ejection fraction of 50%, fractional shortening of 21%, and left ventricular diastolic and systolic diameter of 3.3 and 2.7 cm, respectively, without mitral valve regurgitation (Figs 2 and 3).
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Fig 1. Intraoperative photograph. The lateral wall of the left ventricle has been excised as well as the apical septum. Mild subendocardial fibroelastosis of the left ventricle is evident. (AC = aortic cannula; IVS = interventricular septum; LV = left ventricular free wall; RV = right ventricular free wall; VC = venous cannula.)
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Fig 2. Two-dimentional echocardiogram showing (A) preoperative and (B) postoperative left ventricular diameter at the time of mitral valve closure. (Ao = aorta; IVS = interventricular septum; LA = left atrium; LV = left ventricle; MV = mitral valve; PLVW = posterior left ventricular wall.)
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Fig 3. (A) Preoperative and (B) postoperative M-mode echocardiographic tracings. (IVS = interventricular septum; LV = left ventricular wall; PLVW = posterior left ventricular wall.)
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Slow deterioration in left ventricular function secondary to endocardial fibroelastosis and development of nonfixed pulmonary hypertension led to orthotopic cardiac transplantation 12 months later. The child is alive and well 2 years after transplantation.
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Comment
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Idiopathic dilative cardiomyopathy (IDC) is one of the most frequent indications for cardiac transplantation in the pediatric age group [1, 2]. According to the registry of the International Society for Heart and Lung Transplantation, IDC was the indication for cardiac transplantation in 40.9% of patients between the ages of 0 and 15 years [3]. The natural history of this disease is often characterized by progressive deterioration, in particular for children beyond 2 years of age [4, 5]. Diminished myocardial contractility in the setting of IDC is perhaps the one variable most frequently associated with poor outcome [2, 6], and should prompt evaluation for early transplantation or other interventions. Cardiac transplantation, eventually "bridged" by a ventricular assist device or extracorporeal membrane oxygenation, is currently the only viable long-term therapeutic option for IDC once medical therapy has failed. The scarce availability of organ donors makes the search for alternative sources imperative. Partial left ventriculectomy, as recently reported by Batista and associates, aims at restoring the physiologic ratio between myocardial mass and left ventricular diameter, leading to improvement in cardiac performance [7]. This report represents a unique case of cardiac volume reduction performed in an infant. The patient’s hemodynamic improvement, as noted by comparison of pre- and postoperative M-mode and D-mode echocardiograms, is shown in Figures 2 and 3. Decrease in systolic and diastolic left ventricular diameter, as well as improved contractility, are evident. In the case reported, we observed a very pronounced hyperdynamic state in the early postoperative period, characterized by marked systolic hypertension and mental obtundation deteriorating into a true comatose state, eventually resolving. This response appears strikingly similar to that not infrequently observed after transplantation of oversized donor hearts, the so-called "big-heart syndrome" [8]. This pathophysiologic similarity points to calcium antagonist or beta-blockade therapy as potentially beneficial in these patients, to avoid neurological sequelae.
In conclusion, partial left ventriculectomy may be a bridging technique or, perhaps, even a definitive treatment in those patients with stable but symptomatic cardiomyopathy or those with deteriorating disease who, heretofore, depend on organ transplant as their only hope for survival. Myocardial reduction may also benefit those patients with etiologically proved dilated cardiomyopathy (myocarditis, metabolic, toxic, etc) who fail to improve with time. Accurate patient selection will be of utmost importance, perhaps reserving this treatment modality for those patients who have the least likelihood of improvement over time, or in whom cardiac transplantation may be contraindicated.
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References
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Dec G.W., Fuster V. Idiopathic dilated cardiomyopathy. N Engl J Med 1994;331:1564-1575.[Free Full Text]
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Chan DP, Allen HD. Dilated congestive cardiomyopathy. In: Moss AJ, Adams FH, eds. Heart disease in infants, children and adolescents, 5th ed, vol 2. Baltimore: Williams & Wilkins, 1995:1365–81.
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Hosenpund J.D., Novick R.J., Bennett L.E., Keck B.M., Fiol B., Daily O.P. The registry of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 1996;15:655-674.[Medline]
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Griffin M.L., Hernandez A., Martin T.C., Goldring D., Bolman R.M., Spray T.L., et al. Dilated cardiomyopathy in infants and children. J Am Coll Cardiol 1988;11:139-144.[Abstract]
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Lewis A.B., Chabot M. Outcome of infants and children with dilated cardiomyopathy. Am J Cardiol 1991;68:365-369.[Medline]
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Batista R.J., Santos J.L., Takeshita N., Bocchino L., Lima P.N., Cuhna M.A. Partial left ventriculectomy to improve left ventricular function in end stage heart disease. J Cardiol Surg 1996;11:96-97.
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Reichart B. Size matching in heart transplantation. J Heart Lung Transplant 1992;11(4 Pt 2):S199-S202.[Medline]
Accepted for publication August 12, 1999.
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Abstract
Introduction
