Project description:Postpartum papillary muscle rupture (PMR) is extremely uncommon and tolerated poorly with limited management options other than emergency surgical intervention. This case demonstrates the challenges of postpartum PMR in a young woman with unrecognized vascular Ehlers-Danlos syndrome and highlights the importance of preconception screening of cardiovascular disease. (Level of Difficulty: Beginner.).
Project description:Cardiac involvement in familial amyloid polyneuropathy consists of arrhythmias, conduction disturbances, and heart failure. To our knowledge, heart rupture has never been described in association with this condition. We report the case of a 62-year-old man with a 6-year history of refractory familial amyloid polyneuropathy who underwent liver transplantation. The operation was complicated by severe hypotension because the neuropathy involved the autonomic system. Perioperatively, the patient had a myocardial infarction, and during the next 10 days, a complete interventricular septal rupture developed, resulting in a systemic-to-pulmonary shunt. Coronary angiographic findings were normal. However, the shunt caused unstable hemodynamics, resulting in cardiogenic shock. An attempt to close the rupture percutaneously failed. The patient underwent successful heart transplantation 50 days later. Macroscopic examination of the explanted heart showed thickening of both ventricles, septal rupture, and a gray scar in the interventricular septum around the cavity. Histopathologic examination revealed intramural amyloid angiopathy. Our case shows that heart rupture can occur in patients with familial amyloid polyneuropathy who have no history of obstructive coronary artery disease, perhaps as a result of tissue fragility caused by amyloid angiopathy. Therefore, autonomic disturbances should be regarded with concern and promptly treated in the perioperative period.
Project description:A 64-year-old man presented with several weeks of intermittent irregular palpitations. He had no prior history of cardiac disease, hypertension or syncope. A 12-lead ECG revealed sinus rhythm with premature atrial and ventricular contractions and high QRS voltages consistent with LV-hypertrophy. Cardiac MR revealed asymmetrical septal hypertrophy and marked mid-myocardial hyperenhancement of the interventricular septum.
Project description:An 84-year-old man with hypertension and type 2 diabetes presented with recurrent transient loss of consciousness within 2 hours after dinner at home. Physical examination, electrocardiogram, and laboratory studies were unremarkable except hypotension. Blood pressures were measured in different postures and within 2 hours after meal, but neither orthostatic hypotension nor postprandial hypotension was detected. Further, history taking revealed that the patient was tube-fed with a fluid food pump with an inappropriate rapid infusion rate of 1500 mL per minute at home. He was eventually diagnosed as having syncope due to postprandial hypotension, which was caused by the inappropriate way of tube feeding. The family was educated about appropriate way of tube-feeding and the patient did not develop any episode of syncope during a two-year follow-up. This case highlights the importance of careful history taking in the diagnostic evaluation of syncope and the increased risk of syncope due to postprandial hypotension in the elderly.
Project description:Schwannoma in the retroperitoneal space is rare, and it is extremely rare in patients with no history of neurofibromatosis. We present a case of giant retroperitoneal schwannoma in a 52-year-old man who did not have neurofibromatosis. Because malignant transformation would be extremely rare in this circumstance, close imaging follow-up could avert the necessity for complete resection. The possibility of schwannoma should be considered when evaluating retroperitoneal tumors with the characteristic findings, even if there is no connection between the tumor and the intervertebral foramina.