Texas Heart Institute journal
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Electrical storm is an increasingly common and life-threatening syndrome that is defined by 3 or more sustained episodes of ventricular tachycardia, ventricular fibrillation, or appropriate shocks from an implantable cardioverter-defibrillator within 24 hours. The clinical presentation can be dramatic. Electrical storm can manifest itself during acute myocardial infarction and in patients who have structural heart disease, an implantable cardioverter-defibrillator, or an inherited arrhythmic syndrome. ⋯ Patients who have implantable cardioverter-defibrillators can present with multiple shocks and may require drug therapy and device reprogramming. After the acute phase of electrical storm, the treatment focus should shift toward maximizing heart-failure therapy, performing revascularization, and preventing subsequent ventricular arrhythmias. Herein, we present an organized approach for effectively evaluating and managing electrical storm.
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Congenital heart defects that have a component of right ventricular outflow tract obstruction, such as tetralogy of Fallot, are frequently palliated in childhood by disruption of the pulmonary valve. Although this can provide an initial improvement in quality of life, these patients are often left with severe pulmonary valve insufficiency. Over time, this insufficiency can lead to enlargement of the right ventricle and to the deterioration of right ventricular systolic and diastolic function. ⋯ In patients with preoperative ejection fractions of less than 0.50, mean ejection fractions increased by 0.10. We conclude that pulmonary valve replacement in patients with biventricular dysfunction arising from severe pulmonary insufficiency and right ventricular enlargement can improve left ventricular function. Prospective studies are needed to verify this finding.
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Urgent repair (within 48 hr after diagnosis) of posterior ventricular septal defect in the presence of cardiogenic shock, consequent to acute myocardial infarction, is associated with a very high mortality rate. The use of left ventricular mechanical support devices has the potential to impart hemodynamic stability and to delay surgical treatment until such time as scar tissue forms around the defect, sufficient to hold a suture patch. From May 2004 through July 2007, 5 patients who were in cardiogenic shock as a consequence of acute posterior ventricular septal defect underwent early implantation of a transfemoral microaxial Impella® Recover® LP 5.0 Support System as mechanical support (bridge to surgery). ⋯ Although this is a retrospective study of a very small patient population, without benefit of a control group, it is the first report of its kind. This initial experience using the Impella Recover 5.0 in cases of cardiogenic shock due to posterior ventricular septal defect suggests that this conservative approach is a feasible and safe way to improve hemodynamic conditions and delay surgery. Further clinical experience is needed to confirm these early results.
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Varicella (chickenpox), a common childhood infection caused by the varicella-zoster virus, is self-limiting and usually benign. Although atypical manifestations of the virus are occasionally seen, it rarely presents with cardiovascular sequelae. Cardiovascular complications of varicella can include pericarditis, myocarditis, or endocarditis. ⋯ In 2 weeks, he was asymptomatic, and at 6 months, he was doing well and had normal electrocardiographic and echocardiographic results. To our knowledge, cardiac enzyme elevations to these levels have not been reported in cases of cardiovascular sequelae of varicella. We discuss the diagnostic challenges of this atypical case and suggest that clinicians be aware that varicella disease is most often, but not always, benign.
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Primary pulmonary artery sarcoma is a rare tumor that is highly fatal. It can be misdiagnosed as acute or chronic pulmonary thromboembolic disease. Herein, we report the case of a 22-year-old woman with a preoperative diagnosis of pulmonary embolism and superior vena caval thrombosis. ⋯ Surgical resection of the tumor and reconstruction of the central pulmonary arteries, followed by adjuvant chemotherapy, relieved the clinical symptoms. The patient remained free of cancer at 14 months postoperatively. We believe that this is the 1st report of a primary pulmonary artery sarcoma that extended retrograde into the superior vena cava.