Arrhythmia risk and ß-blocker therapy in pregnant women with long QT syndrome.

BACKGROUND: Pregnancy is one of the biggest concerns for women with long QT syndrome (LQTS). OBJECTIVES: This study investigated pregnancy-related arrhythmic risk and the efficacy and safety of ß-blocker therapy for lethal ventricular arrhythmias in pregnant women with LQTS (LQT-P) and their babies. METHODS: 136 pregnancies in 76 LQT-P (29±5 years old; 22 LQT1, 36 LQT2, one LQT3, and 17 genotype-unknown) were enrolled. We retrospectively analysed their clinical and electrophysiological characteristics and pregnancy outcomes in the presence (BB group: n=42) or absence of ß-blocker therapy (non-BB group: n=94). RESULTS: All of the BB group had been diagnosed with LQTS with previous events, whereas 65% of the non-BB group had not been diagnosed at pregnancy. Pregnancy increased heart rate in the non-BB group; however, no significant difference was observed in QT and Tpeak-Tend intervals between the two groups. In the BB group, only two events occurred at postpartum, whereas 12 events occurred in the non-BB group during pregnancy (n=6) or postpartum period (n=6). The frequency of spontaneous abortion did not differ between the two groups. Fetal growth rate and proportion of infants with congenital malformation were similar between the two groups, but premature delivery and low birthweight infants were more common in those taking BB (OR 4.79, 95% CI 1.51 to 15.21 and OR 3.25, 95% CI 1.17 to 9.09, respectively). CONCLUSIONS: Early diagnosis and ß-blocker therapy for high-risk patients with LQTS are important for prevention of cardiac events during pregnancy and the postpartum period, and ß-blocker therapy may be tolerated for babies in LQT-P cases.

Stent-assisted coil embolization of coronary artery aneurysm.

Coronary artery aneurysms are uncommon diseases with potential complications including rupture and ischemia from embolic events or thrombosis. No consensus has been established regarding the optimal therapy for coronary artery aneurysms. Percutaneous catheter-based treatments using membrane-covered stents and coil embolization have been described. However, only few reports of stent-assisted coil embolization for coronary artery aneurysms have been published to date. Therefore, we report a case of coronary artery aneurysm successfully treated with stent-assisted coil embolization.

Coronary arterial wall disruption and intramural hematoma in a patient with coronary spastic angina.

Acute myocardial infarction is sometimes complicated in patients with coronary spastic angina. The mechanisms are known to be plaque rupture and thrombosis induced by spasm, and reduced coronary flow due to prolonged spasm. We describe the case of a 45-year-old woman with coronary spastic angina who had a complication of an acute myocardial infarction. A specimen obtained with thrombectomy was the disrupted coronary artery wall accompanied by massive intramural hemorrhage. The cause of the acute myocardial infarction was thought to be an embolism of the coronary arterial wall that was disrupted by spasm and intramural hemorrhage.

Sirolimus-eluting stent implantation for ostial stenosis of left main coronary artery after Bentall operation in aortitis syndrome.

We describe a 66-year-old woman with aortitis syndrome, successfully treated with percutaneous coronary intervention using sirolimus-eluting stent (SES) for ostial stenosis of left main coronary artery after Bentall operation. At one-year follow-up, she had no evidence of restenosis and no clinical events. Stent implantation with SES may be useful for ostial left main coronary stenosis after Bentall operation in selected patients with aortitis syndrome.

Left atrial myxosarcoma with previously detected intestinal metastasis.

Primary cardiac myxosarcoma is a rare disease; it is exceedingly rare for symptoms of systemic metastasis to precede diagnosis of the primary cardiac tumor. We describe the case of a previously healthy 60-year-old man with left atrial myxosarcoma, who had first presented with jejunal intussusception due to intestinal polyposis. Three months after resection of the jejunum, the patient experienced cerebral infarction and pulmonary edema. Further physical evaluation, which included echocardiography for the 1st time, revealed a mass in the left atrium that protruded through the mitral valve into the left ventricle. At emergency cardiac surgery, we found that the tumor involved multiple sites of the left atrium, the pulmonary veins, and the mitral anterior leaflet. Two months after surgery, the patient died of massive cerebral hemorrhage. Necropsy disclosed multiple recurrences of the cardiac myxosarcoma and widespread metastatic lesions. The intestinal polyps that had been resected originally were diagnosed, on retrospective histopathologic examination, as metastases of the myxosarcoma. In this unusual case, the metastatic lesions were the 1st clinical manifestations of a malignant cardiac tumor.

Mitral valve surgery in a patient with spinal progressive muscular atrophy: report of a case.

We describe our experience in treating a 69-year-old man with spinal progressive muscular atrophy (SPMA), who underwent a mitral valve replacement. He was admitted for dyspnea, and surgery was indicated for severe mitral insufficiency associated with inferior myocardial infarction. He had been aware of muscle weakness and received a diagnosis of SPMA 18 years previously. Worsening muscle atrophy had led to the need for him to use a wheelchair in his daily life. A preoperative examination revealed markedly reduced pulmonary function (% volume capacity = 44.8%). Because of an acute exacerbation of heart failure, the patient underwent an urgent mitral valve replacement with a 27-mm pericardial bioprosthesis. Although it took 42h to wean him from the mechanical ventilation and he suffered from pulmonary atelectasis after extubation, he was discharged from our hospital in a wheelchair 16 days after surgery. Respiratory management with bilevel positive airway pressure was thus found to be quite useful for patients with neuromuscular disease.

Constrictive pericarditis caused by calcification and organized hematoma 30 years after cardiac surgery.

A 54-year-old man, who had undergone atrial septal defect (ASD) closure 30 years previously, was admitted for exertional dyspnea and chest oppression. He presented with right pleural effusion and hepatomegaly. Hemodynamic characteristics were consistent with constrictive pericarditis caused by multiple cystic lesions anterior to the main pulmonary artery and right ventricle, and severe calcification over the posterior and diaphragmatic sides of the heart. Magnetic resonance imaging was useful for differential diagnosis of the cystic mass and at surgery, it was revealed that the cystic lesions were old hematoma without cells. Pericardiectomy and removal of the calcification were performed safely using an ultrasonic scalpel, without cardiopulmonary bypass, resulting in hemodynamic improvement and relief of his symptoms.