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Severe Pulmonary Hypertension (severe + pulmonary_hypertension)
Selected AbstractsEarly and Midterm Results of an Alternative Procedure to Homografts in Primary Repair of Truncus Arteriosus CommunisCONGENITAL HEART DISEASE, Issue 3 2010Pedro Curi-Curi MD ABSTRACT Background., Repair of truncus arteriosus communis (TAC) in the neonatal and early infant period has become a standard practice. We report our experience on primary repair of TAC with a bovine pericardial-valved woven Dacron conduit as an alternative procedure to homografts, with a focus on early and midterm results. Methods., From January 2001 to December 2007, 15 patients with mean age 1.5 years (range 3 months to 8 years), underwent primary repair of simple TAC. Cases with cardiogenic shock, complex-associated cardiac lesions, or adverse anatomy of the truncal valve were excluded. The Collett and Edwards anatomical type classification of TAC was as follows: type I, 13 (87%); and type II, 2 (13%). Right ventricular outflow tract was reconstructed in all the cases with a bovine pericardial-valved woven Dacron conduit. Results., Overall mortality was 6.6% (1 death due to severe pulmonary hypertension). At a mean follow-up of 31 months (range 6,51), there were no deaths (5-year actuarial survival 93.4%). Out of the 14 midterm survivors, three developed stenosis of the pericardial-valved woven Dacron conduit, but only one underwent interventional procedure including percutaneous balloon dilation with stenting for associated left pulmonary artery hypoplasia. The rate of patients with no surgical or percutaneous reinterventions performed because of obstruction of the right ventricular outflow tract reconstruction in the midterm (5 years) was 86%. Conclusions., Truncus arteriosus communis repair with a bovine pericardial-valved woven Dacron conduit can be performed with a very low perioperative mortality and satisfactory midterm morbidity, favorably compared with that reported for the use of homografts. Interventional cardiac catheterization may delay the time of reoperation for inevitable conduit replacement due to stenosis. [source] Isolated congenital spleen agenesis: A rare cause of chronic thromboembolic pulmonary hypertension in an adultRESPIROLOGY, Issue 6 2008Fumiyuki TAKAHASHI Abstract: This report describes a case of isolated congenital spleen agenesis complicated by chronic thromboembolic pulmonary hypertension (CTPH) in a 44-year-old female patient. The patient had increasing exertional dyspnoea and thrombocytosis. An echocardiogram showed severe pulmonary hypertension and right ventricular hypertrophy, and contrast-enhanced chest CT revealed multiple thromboemboli within both pulmonary arteries. A perfusion lung scan demonstrated multiple segmental defects and no spleen was detected by abdominal CT, ultrasonography or scintigraphy. Comprehensive clinical examinations disclosed no evidence of a thrombus elsewhere or of an associated malformation such as a cardiac anomaly. Anticoagulation therapy was started, and a perfusion lung scan revealed partial improvement of the hypoperfusion in the right lower lobe. However, repeat echocardiography showed the pulmonary hypertension persisting for 1 year. The multiple segmental defects in the perfusion lung scans were also persistent. Collectively, a diagnosis of CTPH with isolated congenital spleen agenesis was established. This is the first documented case of CTPH in an adult with isolated congenital asplenia. Although congenital spleen agenesis is a rare condition, this case report suggests that this possibility should be considered when a diagnosis of CTPH and thrombocytosis is made. [source] Failure of intravenous immunoglobulin to prevent congenital heart block: Findings of a multicenter, prospective, observational studyARTHRITIS & RHEUMATISM, Issue 4 2010C. N. Pisoni Objective Congenital heart block (CHB) is presumed to be caused by transplacental passage of maternal immunoglobulin against Ro and La ribonucleoproteins. The recurrence rate in subsequent pregnancies following the birth of a child with CHB is ,19%. The purpose of this study was to determine whether intravenous immunoglobulin (IVIG) therapy could prevent the development of CHB in the fetuses of high-risk pregnant women. Methods A total of 24 pregnancies in 22 women who had a previous pregnancy in which CHB developed, were over the age of 18 years, were <12 weeks pregnant, and had anti-Ro, anti-La, or both antibodies were monitored in this multicenter, prospective, observational study. Fifteen patients received infusions of IVIG. The 9 pregnancies in the remaining 7 patients served as controls. IVIG was administered at a dose of 400 mg/kg at weeks 12, 15, 18, 21, and 24 of pregnancy. Echocardiograms were performed at least every 3 weeks from week 15 to week 30 of gestation. Electrocardiograms were obtained at birth. The outcome measure was the development of third-degree CHB detected by fetal echocardiogram. Results CHB developed in 3 babies among the 15 pregnancies in the treatment group (20%) and in 1 baby among the 9 pregnancies in the control group (11%). CHB was detected at weeks 18, 23, and 26, respectively, in the 3 babies in the treated group and at week 19 in the baby in the control group. Three of the affected pregnancies ended in termination; 2 for reasons related to the fetal disease and 1 for reasons related to both maternal (severe pulmonary hypertension) and fetal disease (at 21 weeks of gestation). Conclusion IVIG at the dose and frequency used in this study was not effective as prophylactic therapy for CHB in high-risk mothers. [source] Left main coronary artery compression from pulmonary artery enlargement due to pulmonary hypertension: A contemporary review and argument for percutaneous revascularization,CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS, Issue 4 2010Michael S. Lee MD Abstract Extrinsic compression of the left main coronary artery by an enlarged pulmonary artery is an increasingly recognized and potentially reversible cause of angina and left ventricular dysfunction in patients with pulmonary hypertension. The diagnosis of extrinsic left main coronary artery compression requires a high index of suspicion and should be considered in patients with severe pulmonary hypertension who experience angina. Coronary angiography with intravascular ultrasound is the gold standard for diagnosis of this condition, though cardiac computed tomography and magnetic resonance angiography allow for noninvasive means of screening. The optimal treatment is debatable, but percutaneous coronary intervention appears to be a feasible, safe, and effective treatment option for patients with extrinsic compression of the left main coronary artery from pulmonary artery enlargement. Given the high risk of postoperative right ventricular failure and mortality observed with surgical revascularization in these patients, we recommend that physicians recognize percutaneous coronary intervention as the preferred revascularization strategy for selected patients with extrinsic compression of the left main coronary artery due to pulmonary hypertension. © 2010 Wiley-Liss, Inc. [source] Rapid assessment and safe management of severe pulmonary hypertension with milrinone during orthotopic liver transplantationCLINICAL TRANSPLANTATION, Issue 4 2010Kyota Fukazawa Fukazawa K, Poliac LC, Pretto EA. Rapid assessment and safe management of severe pulmonary hypertension with milrinone during orthotopic liver transplantation. Clin Transplant 2010: 24: 515,519. © 2009 John Wiley & Sons A/S. Abstract:, The incidence of porto-pulmonary hypertension (PPHN) in patients with end stage liver disease is 8.5%. Evidence indicates that proceeding with orthotopic liver transplantation (OLT) in patients diagnosed with severe PPHN (mean pulmonary artery pressure [mPAP] > 45 mmHg) at the time of OLT surgery is associated with high perioperative mortality. We describe a case of severe PPHN that was diagnosed by right heart catheterization at the time of surgery. We quickly determined the reversibility of PPHN with a bolus of milrinone and proceeded with OLT. Further episodes of pulmonary hypertension were successfully managed with continuous milrinone infusion and transesophageal echocardiography monitoring. Reversibility via vasodilator trial after identification of high pulmonary artery pressures (PAP) may be an important indication of the feasibility of OLT. Milrinone may be useful for the rapid identification of the reversibility of high PAP and may be an effective agent to control abrupt increases in PAP during OLT. [source] | ||||||||||