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1/56. Profound hypoxemia during treatment of low cardiac output after cardiopulmonary bypass.

    PURPOSE: To illustrate the multiple causes of hypoxemia to be considered following cardiopulmonary bypass and how therapy given to improve oxygen delivery may have contributed to a decrease in arterial oxygen saturation to life-threatening levels. CLINICAL FEATURES: A 61 yr old man with severe mitral regurgitation and chronic obstructive lung disease underwent surgery for mitral valve repair. A pulmonary artery catheter with the capacity to measure cardiac output and mixed venous oxygen saturation (SvO2) continuously was used. Two unsuccessful attempts were made to repair the valve which was finally replaced, requiring cardiopulmonary bypass of 317 min. dobutamine 5 micrograms.kg-1.min-1 and sodium nitroprusside 1 microgram.kg-1.min-1 were used to increase cardiac output. Soon after, the SvO2 decreased progressively from 55 to 39%. The patient became cyanotic with a PaO2 of 39 mmHg. sodium nitroprusside was stopped and amrinone 100 mg bolus followed by 10 micrograms.kg-1.min-1 was given in addition to adding PEEP to the ventilation. With these measures PaO2 could be maintained of safe levels but PEEP and high inspired oxygen concentrations were needed postoperatively until the trachea could be extubated on the third postoperative day. CONCLUSION: The profound hypoxemia in this case was likely due to a combination of intra- and extrapulmonary shunt, both augmented by sodium nitroprusside. The desaturation of mixed venous blood amplified the effect of these shunts in decreasing arterial oxygen saturation. The interaction of these factors are analyzed in this report.
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2/56. Expeditious diagnosis of primary prosthetic valve failure.

    Primary prosthetic valve failure is a catastrophic complication of prosthetic valves. Expeditious diagnosis of this complication is crucial because survival time is minutes to hours after valvular dysfunction. The only life-saving therapy for primary prosthetic valve failure is immediate surgical intervention for valve replacement. Because primary prosthetic valve failure rarely occurs, most physicians do not have experience with such patients and appropriate diagnosis and management may be delayed. A case is presented of a patient with primary prosthetic valve failure. This case illustrates how rapidly such a patient can deteriorate. This report discusses how recognition of key findings on history, physical examination, and plain chest radiography can lead to a rapid diagnosis.
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3/56. endocarditis attributable to group A beta-hemolytic streptococcus after uncomplicated varicella in a vaccinated child.

    Varicella is generally a benign, self-limited childhood illness; however, severe, life-threatening complications do occur. A live, attenuated vaccine exists to prevent this illness, but controversy remains concerning the need to vaccinate children for what is generally a benign, self-limited disease, although more states are currently recommending this vaccine. We report a previously healthy 3-year-old who developed varicella 6 months after vaccination with no apparent skin superinfections, who subsequently developed group A beta-hemolytic streptococcus (GABHS) bacteremia resulting in endocarditis of a normal heart valve. We are unaware of previous reports of endocarditis related to GABHS after varicella. After developing a harsh, diastolic murmur that led to an echocardiogram, aortic valve endocarditis was diagnosed. A 6-week course of intravenous penicillin g was administered. Two weeks after the initiation of therapy, the diastolic murmur was harsher, and echocardiography revealed a large vegetation on the posterior leaflet of the aortic valve, with severe aortic insufficiency and a dilated left ventricle. The patient subsequently developed congestive heart failure requiring readmission and aggressive management. One month after the initial echocardiogram, a repeat examination revealed worsening aortic regurgitation and mitral regurgitation. The patient received an additional 4 weeks of intravenous penicillin and gentamicin followed by aortic valve replacement using the Ross procedure. Our patient, the first reported case of bacteremia and endocarditis from GABHS after varicella, illustrates the need for the health care practitioner to consider both common and life-threatening complications in patients with varicella. While cellulitis, encephalitis, and septic arthritis may be readily apparent on physical examination and commonly recognized complications of varicella, the possibility of bacteremia without an obvious skin superinfection should also be entertained. The case we report is unique in that the patient had normal immune function, had been previously vaccinated, and developed a rare complication of varicella-endocarditis-in a structurally normal heart with a previously unreported pathogen. Although a child may have been vaccinated against varicella, the chance of contracting the virus still exists and parents should be informed of this risk. group A beta-hemolytic streptococcus, endocarditis, varicella, Varivax, complications of varicella.
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4/56. Critical left ventricular outflow tract obstruction due to accessory mitral valve tissue.

    Left ventricular outflow tract (LVOT) obstruction due to anomalous tissue tag arising from the mitral valve is a rare congenital cardiac anomaly. It generally becomes symptomatic during the first decade of life as exercise intolerance, chest pain, or syncope at effort. To date, only a few cases of critical systemic obstruction due to isolated mitral valve anomaly in neonates have been reported. We report the case of a neonate who was a few hours old and was referred in severe clinical condition due to critical left ventricular outflow obstruction resulting from an anomalous tissue tag of mitral valve origin.
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5/56. Left atrial aneurysm.

    Aneurysms of the left atrium are rare abnormalities. They can be congenital or acquired. Whereas a true congenital aneurysm presents as isolated pathology, inflammatory or degenerative processes involving the endocardium are associated with the acquired type. The clinical records of 2 patients with the diagnosis of left atrial aneurysm were reviewed, along with the surgical strategies, current literature, and patient outcomes. Because of the risk of life-threatening complications, surgery is recommended even in asymptomatic cases. Resection and mitral valvuloplasty should be the treatment of choice.
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6/56. Emergency pulmonary autograft mitral valve replacement in a child.

    Mitral valve replacement in small children imposes significant clinical difficulties because of the relatively small mechanical prosthetic valves required and the need for lifelong anticoagulation therapy. A child weighing 10.4 kg presented with thrombosis of her 19-mm mechanical mitral prosthesis 4 weeks after implantation despite appropriate oral anticoagulation therapy. An emergency mitral valve replacement with a pulmonary autograft was successfully performed with encouraging short-term results.
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7/56. Severe mitral regurgitation due to mitral valve prolapse associated with Bland-White-Garland syndrome.

    Bland-White-Garland syndrome refers to the rare congenital cardiac abnormality whereby the left coronary artery arises from the pulmonary artery. The natural history of this condition is highly variable, ranging from death in early infancy to asymptomatic adult survival. It is sometimes diagnosed in adults with mitral regurgitation thought to be of ischaemic origin. We report a case of a 29-year-old man with Bland-White-Garland syndrome and concomitant mitral valve prolapse, and review the literature on the appropriate investigations and management of this abnormality. Recognition and diagnosis of this condition is important because of the potentially life-threatening complications, which may be prevented by surgical intervention.
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8/56. Mitral stenosis and regurgitation with systemic lupus erythematosus and antiphospholipid antibody syndrome.

    Cases of valvular disease accompanied by systemic lupus erythematosus and antiphospholipid antibody syndrome are uncommon. Here, we present a surgical case of mitral stenosis and regurgitation with systemic lupus erythematosus and antiphospholipid antibody syndrome. Mitral valve replacement was performed, and the postoperative course was uneventful. Pathohistological findings of the mitral valve showed degenerative change due to chronic inflammation, proliferative fibrous change and calcification without thrombus formation.
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9/56. Cardiac complications in children following infection with varicella zoster virus.

    Infection with varicella zoster virus, leading to chicken pox in susceptible hosts, is usually a benign self-limiting disease conferring immunity in those affected. Cardiac complications are rare, but when present may lead to severe morbidity or mortality. We have recently encountered three children, all of whom developed significant cardiac complications secondary to infection with varicella. myocarditis has long been associated with such infection. The pathological mechanism is presumed similar to other cardiotropic viruses, where both direct cytopathic and secondary auto-immune effects contribute to myocardial cellular destruction and ventricular dysfunction. Complications include arrhythmias and progression to dilated cardiomyopathy. pericarditis, and secondary pericardial effusion, related to infection with the virus is most commonly associated with secondary bacterial infiltration. Both cardiac tamponade and chronic pericardial constriction may result. endocarditis complicating varicella has only been described in the last fifteen years, and is associated with the emergence of virulent strains of both streptococcus and staphylococcus, the two organisms most commonly associated with endocarditis. The exact mechanism by which varicella causes secondary bacterial endocarditis remains unclear. Whilst cardiac complications of infection with the varicella zoster virus are rare, the resulting complications are potentially life threatening. Evidence of varicella-induced carditis must be aggressively pursued in any child with signs of acute cardiac decompensation in whom chicken pox is confirmed or suspected.
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10/56. A case of papillary muscle rupture caused by acute myocardial infarction.

    A 63-year-old female was admitted because of cardiogenic shock due to complete rupture of the posterior papillary muscle. The diagnosis was made by echocardiography. She was treated successfully by an emergency replacement of the mitral valve. early diagnosis and surgical treatment are mandatory and life-saving in the case of complete papillary muscle rupture.
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