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1/29. Utility of thallium-201 scintigraphy in detecting right ventricular dysfunction in pulmonary embolism.

    Acute right ventricular dysfunction has been established both as a diagnostic and prognostic indicator in pulmonary embolism. This report illustrates the utility of thallium-201 scintigraphy as an adjunctive noninvasive test in the diagnosis of pulmonary embolism by demonstrating increases in regional right ventricular perfusion and its subsequent resolution with treatment presumably as a result of decreased pressure work.
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2/29. Pulmonary stenosis and severe biventricular dysfunction: improvement following percutaneous valvuloplasty.

    A 15-year-old boy with severe pulmonary stenosis associated with severe right and left ventricular systolic dysfunction is reported. After successful percutaneous pulmonary valvuloplasty, there was an initial and early improvement in right ventricular (RV) function, followed by a delayed and more gradual improvement in left ventricular (LV) function. At long-term follow up, both RV and LV systolic functions were nearly normalized. Several mechanisms may be implicated, including ventricular interdependence, geometric factors, altered compliance and intrinsic alteration in the LV muscle. A delayed, but sustained, improvement in LV systolic function following relief of RV pressure overload suggests that the latter mechanism must have played an important role in the genesis of the LV dysfunction. Pulmonary stenosis associated with severe biventricular dysfunction may be treated primarily by percutaneous pulmonary balloon valvuloplasty with near-total recovery of the ventricular function.
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3/29. Balloon pulmonary valvuloplasty in carcinoid syndrome.

    Half of all patients with carcinoid syndrome develop cardiac involvement. patients who have cardiac involvement have a significantly worse prognosis than those without, and death can occur directly as a result of cardiac involvement. A case of carcinoid syndrome in a 38 year old woman with lesions in the liver, who presented with right sided valvar abnormalities, a dilated right ventricle, and right ventricular pressure overload, is presented. In order to palliate the patient's symptoms and to decrease right sided pressures before major abdominal surgery, balloon pulmonary valvuloplasty was performed at the time of cardiac catheterisation. This resulted in a reduction in the pulmonary gradient and right ventricular pressure. Following the procedure, the patient's symptoms were completely relieved. She went on to laparotomy where the lesions in the liver were excised without complication.
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4/29. Arterial switch operation after left ventricular retraining in the adult.

    Retraining the morphological left ventricle in transposition of the great arteries has been successfully reported in infancy, while older age seems to be a contraindication. A 23-year-old woman with inverted question markS,D,D inverted question mark transposition of the great arteries and ventricular septal defect developed severe right systemic ventricular dysfunction 22 years after Mustard procedure and ventricular septal defect closure. Hemodynamic investigation revealed moderate pulmonary hypertension and preserved left ventricular function. A pulmonary artery band was applied to obtain a left-right ventricular pressure ratio of 0.91. Her postoperative course was characterized by biventricular failure, treated effectively with inotropic support. Six months later, she underwent a Mustard baffle takedown and arterial switch procedure. Her postoperative course was uneventful. She was discharged home on postoperative day 15. At 24-months follow-up, she is in excellent clinical condition; echocardiographic evaluation shows good left ventricular function (ejection fraction: 0.69) with left ventricular volume within normal limits (70 ml/m2). Our experience demonstrates that, despite adult age, a staged arterial switch operation can be performed successfully in selected patients when left ventricular function is preserved.
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5/29. norepinephrine can be useful for the treatment of right ventricular failure combined with acute pulmonary hypertension and systemic hypotension. A case report.

    A 48-year-old woman who underwent emergency cardiac surgery for removal of a thrombus partially occluding the mitral valve, developed pulmonary hypertension right ventricular failure and systemic hypotension, in the immediate postoperative period, a clinical condition not well controlled by high doses of epinephrine and dobutamine. The addition of a continuous infusion of norepinephrine in incremental dosages, caused the rise in cardiac index accompanied before by the reduction in the pulmonary pressure and the stability in the systemic pressure, than by the further reduction in the pulmonary pressure and the increase in the systemic pressure. The conclusion is drawn that norepinephrine is useful in the treatment of right ventricular failure which follows a condition of acute pulmonary hypertension, because the improvement of cardiac performance established without adverse effects on the pulmonary pressures whose values on the contrary progressively declined.
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6/29. Systemic right ventricular failure after atrial switch operation: midterm results of conversion into an arterial switch.

    BACKGROUND: Failure of the systemic right ventricle after atrial switch operation can be treated by conversion into an arterial switch operation. methods: Four patients, age 38 to 59 months, presented with right ventricular failure after Senning operation and ventricular septal defect closure. One patient had elevated left ventricular pressure; in the other three patients the left ventricle was retrained to a left ventricular/right ventricular pressure ratio of 0.8 or greater by pulmonary artery banding in 12 to 24 months. RESULTS: Postoperative course after arterial switch operation was prolonged, but clinical condition was good at discharge. Fractional shortening ranged from 20% to 28%. Trace-to-moderate aortic regurgitation was present; only 1 patient had preserved sinus rhythm. After a mean follow-up of 43.5 months 1 patient had died due to left ventricular dysfunction. The survivors are in new york Heart association functional class I to II. Fractional shortening has improved (29% to 37%); aortic regurgitation has not increased. No patient has undisturbed sinus rhythm. CONCLUSIONS: Conversion of an atrial into an arterial switch is an alternative to cardiac transplantation in childhood. However, the procedure is demanding. Long-term morbidity is caused by rhythm disturbances. aortic valve performance and left ventricular function require close observation.
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7/29. Decreased plasma neurohormones and improved cardiac performance after surgical treatment of chronic pulmonary embolism.

    The findings of this case report suggest that quantitative assessment of plasma neurohormones and magnetic resonance imaging functional parameters in patients with right ventricular pressure overload due to chronic pulmonary embolism might be used as indicators for right ventricular function before and after intervention. Monitoring of changes in these parameters may provide quantitative follow-up of right ventricular function in these patients.
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8/29. Acute right ventricular failure--from pathophysiology to new treatments.

    The right ventricle (RV) provides sustained low-pressure perfusion of the pulmonary vasculature, but is sensitive to changes in loading conditions and intrinsic contractility. Factors that affect right ventricular preload, afterload or left ventricular function can adversely influence the functioning of the RV, causing ischaemia and right ventricular failure (RVF). As RVF progresses, a pronounced tricuspid regurgitation further decreases cardiac output and worsens organ congestion. This can degenerate into an irreversible vicious cycle.The effective diagnosis of RVF is optimally performed by a combination of techniques including echocardiography and catheterisation, which can also be used to monitor treatment efficacy. Treatment of RVF focuses on alleviating congestion, improving right ventricular contractility and right coronary artery perfusion and reducing right ventricular afterload. As part of the treatment, inhaled nitric oxide or prostacyclin effectively reduces afterload by vasodilating the pulmonary vasculature. Traditional positive inotropic drugs enhance contractility by increasing the intracellular calcium concentration and oxygen consumption of cardiac myocytes, while vasopressors such as norepinephrine increase arterial blood pressure, which improves cardiac perfusion but increases afterload. A new treatment, the calcium sensitiser, levosimendan, increases cardiac contractility without increasing myocardial oxygen demand, while preserving myocardial relaxation. Furthermore, it increases coronary perfusion and decreases afterload. Conversely, traditional treatments of circulatory failure, such as mechanical ventilation and volume loading, could be harmful in the case of RVF. This review outlines the pathophysiology, diagnosis and treatment of RVF, illustrated with clinical case studies.
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9/29. A 38-year-old man with pulmonary hypertension, who had undergone atrial septal closure 26 years previously.

    The patient was a 38-year-old man. He underwent atrial septal closure at the age of 12 years at Yokohama City University Hospital, when he already had pulmonary vascular change and reduced left-to-right shunt with Qp/Qs of 1.55 and pulmonary artery pressure (PA) of 56/22 mmHg. Thereafter, he enjoyed running and skiing without any symptoms up until 32 years of age, when he developed syncope due to severe pulmonary hypertension and atrial flutter. PA was 116/57 mmHg and mRA was 13 mmHg on cardiac catheterization. He developed right heart failure and was referred to Keio University Hospital on May 12th, 2001. Home intravenous prostacyclin infusion therapy was introduced in addition to treatment for right heart failure. echocardiography revealed a residual interatrial shunt (from right to left). He recovered and was discharged. His condition worsened again and he was readmitted to our hospital with chief complaint of visual disturbance due to digoxin intoxication, in addition to right heart failure. Despite aggressive treatment, he died of severe pulmonary hypertension, right heart failure and congestive hepatic failure on December 10th, 2001. The differential diagnosis, pathophysiology and necessary treatment of pulmonary hypertension are discussed in this paper. The clinical diagnosis was Eisenmenger syndrome due to atrial septal defect, and the pathological findings were compatible with this.
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10/29. Arterial switch operation after Mustard procedures in adult patients with transposition of the great arteries: is it time to revise our strategy?

    BACKGROUND: After the Mustard or Senning procedure, adults with transposition of the great arteries may have right ventricular failure and require consideration of new therapies. A 2-stage arterial switch operation (ASO) may be performed as an alternative to heart transplantation. This procedure is relatively successful in children, but little is known about the 2-stage ASO in adults. We report our experience in adults undergoing pulmonary arterial banding as the first stage of a planned 2-stage arterial switch procedure after a failed Mustard operation. methods AND RESULTS: Three adult patients with systemic right ventricular failure late after Mustard procedures embarked, through pulmonary artery banding, on a course toward a 2-stage arterial switch at the Toronto General Hospital. Baseline clinical characteristics as well as preoperative hemodynamics were reviewed. Immediate perioperative and postoperative events, hemodynamic measurements, and clinical outcomes were also recorded. Two patients were banded acutely such that their morphologic left ventricular to right ventricular (LV/RV) systolic pressure ratios were >0.65 after the initial banding procedure. The subpulmonary left ventricle failed in both cases. In contrast, the third patient had a more gradual approach to pulmonary artery banding (PAB), with an initial LV/RV pressure ratio of 0.5, which eventually led to a successful conversion to an arterial switch procedure. CONCLUSIONS: Our evidence suggests that in adult patients expected to undergo a 2-stage arterial switch procedure after a failed Mustard operation, acute PAB achieving near-systemic subpulmonary LV pressure leads rapidly to ventricular failure and failure of this treatment strategy. A more gradual approach to PAB may be required to achieve a successful outcome.
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