![]() There are a proportion of patients (4-5%) who progresses to refractory heart failure requiring vasoactive support and heart transplantation as in our case. The patient’s brother was also screened testing genetically positive but phenotypically negative, it means, the absence of structural cardiomyopathy ( Figure 3).Īlthough the clinical course of peripartum cardiomyopathy is generally benign, with recovery of function and ventricular dimensions. In this study, a heterozygosis of variant c.40460_40461insT P.Leu13487Phefs * 6 in the TTN gene (2q31, which encodes the sarcomeric titin protein) was detected in both patients. Considering the presence of dilated cardiomyopathy in two members of the same family, genetic studies by means of next-generation sequencing (Illumina Genome Analyzer II) were conducted. No coronary lesions detected on the heart catheterization. Dilatation and severe left ventricle dysfunction were observed in the echocardiogram. Two years later, the patient’s mother presented with acute heart failure at age 48. In the absence of clinical improvement after three weeks of treatment, the patient was transferred to a specialized medical center for heart transplantation which was successfully performed 20 days after the initiation of extracorporeal membrane oxygenation therapy. Extracorporeal membrane oxygenation system (veno–arterial) was added to the whole therapeutic arsenal and 24 hours later a counterpulsation balloon was placed. Heart catheterization revealed pulmonary capillary pressure 29 mmHg and mean pulmonary artery pressure 35 mmHg accompanied by increased transpulmonary gradient (>12 mmHg ) and cardiac output estimated of 1.6 ml/min. ![]() Cardiac magnetic resonance did not show edema or late enhancement so myocarditis was ruled out. Diuretics, bromocriptin and angiotensin enzyme inhibitors were added. ![]() She needed orotracheal intubation, noradrenaline and levosimendan. In the setting of this clinical picture, the diagnosis of peripatum cardiomyopathy was evoked.The patient clinically progressed to severe acute heart failure and was transferred to the intensive care unit. The echocardiogram revealed dilation and severe dysfunction of left ventricle as well as important mitral and triscupid insufficiency ( Figure 2). Considering a recent delivery as well as the laboratory tests, a thorax CT scan to rule out pulmonary embolism was indicated but it only demonstrated the presence of pleural effusion and vascular congestion. A complete laboratory panel showed increase Troponin T (0,03 ng/dL), proBNP (6000 pg/mL) and D Dimers (>5000 mg/dL). Cardiomegaly and signs of vascular redistribution were found on the chest X-rays. Electrocardiogram showed a sinus tachycardiawith diffuse repolarization changes ( Figure 1). Upon arrival she was tachycardic and oxygen saturation on room air was 91%. She was admitted to the emergency department for progressive dyspnea starting two weeks prior to delivery. 28 year old woman with no previous medical history who recently presented a normal delivery at week 38 of gestation.
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |