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J Thorac Cardiovasc Surg 2005;129:680-681
© 2005 The American Association for Thoracic Surgery

Brief Communications

Acute mitral regurgitation caused by papillary muscle rupture in the immediate postpartum period revealing Ehlers-Danlos syndrome type IV

^a Internal Medicine
^b Medical Genetics,
^c Hôtel-Dieu, and the Coronary Care Unit
^d Department of Cardiovascular Surgery, Hôpital Louis Pradel, Hospices Civils de Lyon, Université Claude Bernard Lyon I, Lyon, France

Received for publication June 17, 2004; revisions received July 13, 2004; accepted for publication July 21, 2004.

^* Address for reprints: Christiane Broussolle, MD, Department of Internal Medicine, Hôtel-Dieu, 1 place de l'Hôpital, 69288 Lyon Cedex 02, France (E-mail: christiane.broussolle@chu-lyon.fr).

The first 20% of the full text of this article appears below.

Ehlers-Danlos syndrome (EDS) type IV, the vascular type, is a rare autosomal dominant inherited disorder of connective tissue resulting from mutation of the COL3A1 gene encoding type III collagen.^1-3 Affected patients often have life-threatening cardiovascular complications.^1-3 We report here a case of EDS type IV diagnosed 1 week after delivery in a patient with a severe mitral regurgitation caused by papillary muscle rupture requiring emergency surgical intervention.

	Clinical summary

 
A 36-year-old female patient was admitted to the intensive care unit with a diagnosis of severe mitral regurgitation caused by papillary muscle rupture. She had a medical history of pneumothorax at birth. One week before, the patient had been delivered spontaneously of her first pregnancy at 37 weeks' gestation. Delivery was complicated by vaginal bleeding from severe perineal tears. Five days later, the patient had respiratory distress with circulatory collapse. Chest radiography showed pulmonary edema. Transesophageal echocardiography . . . [Full Text of this Article]