Anomalous papillary muscles-Implications in the surgical treatment of hypertrophic obstructive cardiomyopathy.

OBJECTIVES: To categorize and assess the functional significance of anomalous papillary muscles in patients undergoing surgical management of obstructive hypertrophic cardiomyopathy. METHODS: We reviewed the records of operations for obstructive hypertrophic cardiomyopathy and identified 73 patients with an anomalous papillary muscle. Anomalous papillary muscles inserting directly into the body of the anterior mitral valve leaflet were classified as type I, those with both direct insertion into the body of the leaflet and attachment to the free edge of the anterior leaflet were categorized as type II, and anomalous papillary muscles inserting into the free edge of the anterior leaflet were grouped as type III. Additionally, we investigated detection rates by preoperative transthoracic echocardiography, intraoperative transesophageal echocardiography, and cardiac magnetic resonance imaging. RESULTS: The mean age of patients was 51.9 ± 12.3 years, and 49.3% were male. The anomalous papillary muscle was classified as type I in 31.5% of patients, type II in 35.6%, and type III in 32.9%. Only type I and type II anomalous papillary muscles contributed to left ventricular outflow tract obstruction. The anomalous papillary muscle was detected on preoperative transthoracic echocardiography in 11% of patients and by intraoperative transesophageal echocardiography in 27.4% of patients. No anomalous papillary muscles were identified on cardiac magnetic resonance imaging. All patients underwent septal myectomy with or without (n = 34) associated excision of the anomalous papillary muscle. Excision of the papillary muscles was more common in patients with type I and II (76.4% and 80.8%, respectively) when compared with type III (4.2%). Ten patients underwent mitral valve repair, and 1 patient had mitral valve replacement. CONCLUSIONS: Papillary muscle abnormalities are important findings in patients with obstructive hypertrophic cardiomyopathy but are not identified preoperatively in the majority of patients. Recognition of anomalous papillary muscles intraoperatively and understanding of the morphologic subtypes are critical to adequate gradient relief and preservation of mitral valve function. The optimum approach involves a transaortic extended septal myectomy associated with the resection of the anomalous papillary muscles in patients with type I and II anatomy.

Intravascular cardiac lipoproteinosis: extrarenal manifestation of lipoprotein glomerulopathy.

Intracapillary lipoprotein thrombi are a distinct histopathologic finding described in the setting of lipoprotein glomerulopathy. The disease is associated with mutations in the apolipoprotein E gene and responds well to lipid-lowering treatments. Lipoprotein glomerulopathy is thought to primarily affect the kidneys, and lipoprotein thrombi have never been described in any other organ. Herein we present the first recognized case with extrarenal manifestations in the form of intravascular cardiac lipoprotein deposition.