Postoperative mitral leaflet rupture in an infant with Loeys-Dietz syndrome.

This report describes an infantile case of Loeys-Dietz syndrome (LDS) with spontaneous mitral leaflet rupture. The patient was diagnosed with a type B interruption of the aortic arch. Bilateral pulmonary artery banding was performed 5 days after birth. On the 53rd day, intra-cardiac repair was performed without valvuloplasty. Although the operation was successful, mitral regurgitation deteriorated at 4 weeks after operation. On the 88th day, a mitral valvuloplasty was performed and a severely ruptured anterior leaflet was observed. Seven days after valvuloplasty, the mitral valve insufficiency again worsened and a fourth operation was performed. Two tears were observed in the anterior and posterior mitral valve leaflets, and a mitral valve replacement was required. Subsequently, the patient was diagnosed with LDS according to gene mutational status. LDS is known to have a poor prognosis with cardiovascular complications, but valve rupture has not been previously reported in other cases.

Aortic arch repair with antegrade selective cerebral perfusion using mild to moderate hypothermia of more than 28°C.

BACKGROUND: The temperature at circulatory arrest during open distal anastomosis is the most significant issue for aortic arch repair. In many institutions, there has been trend toward raising the temperature during circulatory arrest. METHODS: Between 2004 and 2011, 164 consecutive patients underwent aortic arch repair with antegrade selective cerebral perfusion (ASCP) and moderate hypothermia. The patients were divided into two subsets (n = 84 each): group A (circulatory arrest at less than 27.9°C) and group B (at more than 28°C). RESULTS: In group A compared with group B, mean temperature at circulatory arrest was 26° ± 1.0°C vs 29° ± 1.0°C, mean ASCP time was 72 ± 23 minutes vs 67 ± 17 minutes, and mean circulatory arrest time was 47 ± 21 minutes vs 44 ± 13 minutes. The 30-day mortality was 6.1% in both groups. Permanent neurologic deficit occurred in 8 patients (9.8%) in group A and in 5 (6.1%) in group B (p = 0.39). The incidence of renal failure requiring hemodialysis was 14.6% in group A and 3.6% in group B (p = 0.02). Postoperative respiratory failure requiring mechanical ventilation exceeding 3 days occurred in 12.2% of patients in group A and in 7.3% in group B (p = 0.04). CONCLUSIONS: The temperature during ASCP can be safely increased to more than 28°C without increasing the rate of mortality and morbidity. ASCP with moderate hypothermia offered sufficient cerebral and distal organ protection.

Measles encephalitis and acute pancreatitis in a young adult.

Although rare, encephalitis and hepatitis are major complications of measles that are more common in adults than in infants. On the other hand, although several other complications of measles, such as pneumonia and myocarditis, are found in all ages, acute pancreatitis in measles is very rare in both children and adults. We describe a 16-year-old female patient with measles encephalitis who developed acute pancreatitis. The response to steroid therapy was favorable.