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(Circulation. 2000;101:109.)
© 2000 American Heart Association, Inc.

Images in Cardiovascular Medicine

Cantrell’s Syndrome

Left Ventricular Diverticulum in an Adult Patient

Luis Gruberg, MD; Steven A. Goldstein, MD; Albert J. Pfister, MD; Lee H. Monsein, MD; David M. Evans, MD; Martin B. Leon, MD

From the Department of Internal Medicine (Cardiology Division) of the Washington Hospital Center, Washington, DC.

Correspondence to Martin B. Leon, MD, Director and CEO, Cardiology Research Foundation, Suite 4B-1, 110 Irving St NW, Washington, DC 20010. E-mail mbl1{at}mhg.edu

A49-year-old woman underwent cardiac evaluation because of an abnormal ECG. She had undergone multiple surgical procedures during her infancy for congenital right tibial aplasia and polydactyly. At the age of 33 years, she had undergone cardiac evaluation after a questionable episode of supraventricular tachycardia and an abnormal ECG (Figure 1). An echocardiogram revealed left ventricular hypertrophy; coronary angiography and left ventriculography were normal.

View larger version (99K): [in this window] [in a new window]   Figure 1.

A routine follow-up in 1998 with a 2D echocardiogram demonstrated a large apical diverticulum (D) (Figure 2; LV indicates left ventricle; LA, left atrium). Cardiac catheterization with left ventriculography (Figure 3) and MRI (Figure 4) showed a diverticulum originating at the apex of the left ventricle. The patient underwent cardiac surgery with excision of the diverticulum and pericardial patch repair without complications.

View larger version (120K): [in this window] [in a new window]   Figure 2.

View larger version (132K): [in this window] [in a new window]   Figure 3.

View larger version (117K): [in this window] [in a new window]   Figure 4.

Congenital diverticulum of the ventricle is a rare cardiac malformation initially described in 1838.¹ It is usually detected in children and was described in detail by Cantrell et al in 1958 as part of a pentalogy.² The classic clinical picture is characterized by congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. At least 9 patients, all newborn, with associated limb malformations have been described.³ In 30% of the cases, left ventricular diverticulum is not associated with congenital malformations and is referred to as isolated left ventricular diverticulum.

Prognosis depends on the severity of the cardiac and associated malformations. In patients with complex cardiac malformations who undergo surgery during the first days of life, mortality can be as high as 50%. Cardiac rupture, tamponade, sudden death, endocarditis, peripheral embolism, heart failure, and arrhythmia have all been described as complications and causes of death.^{3 4}

Surgical resection with repair of the associated malformations, even in asymptomatic patients, is the treatment of choice to prevent potentially catastrophic complications.

Footnotes

The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.

Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke’s Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.

References

1. O’Bryan. In: Peacock TB, ed. On Malformations of the Human Heart. 2nd ed. London, UK: Churchill & Sons; 1866.
   
2. Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynecol Obstet. 1958;107:602–614.[Medline] [Order article via Infotrieve]
   
3. Vazquez-Jimenez JF, Muehler EG, Daebritz S, Keutel J, Nishigaki K, Huegel W, Messmer BJ. Cantrell’s syndrome: a challenge to the surgeon. Ann Thorac Surg. 1998;65:1178–1185.[Abstract/Free Full Text]
   
4. Walton-Shirley M, Smith SM, Talley D. Left ventricular diverticulum: case report and review of the literature. Cathet Cardiovasc Diagn. 1992;26:31–33.[Medline] [Order article via Infotrieve]
   

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