Melody transcatheter pulmonary valve replacement: a single-center case series in Southeast Asia.

Background: Studies of transcatheter pulmonary valve replacement (TPVR) with the Melody valve have demonstrated good clinical and hemodynamic outcomes. Our study analyzes the midterm clinical and hemodynamic outcomes for patients who underwent Melody valve implantation in Southeast Asia.

Methods: Patients with circumferential conduits or bioprosthetic valves and experiencing post-operative right ventricular outflow tract (RVOT) dysfunction were recruited for Melody TPVR.

Cardiovascular Complications of Down Syndrome: Scoping Review and Expert Consensus.

Cardiovascular disease is a leading cause of morbidity and mortality in individuals with Down syndrome. Congenital heart disease is the most common cardiovascular condition in this group, present in up to 50% of people with Down syndrome and contributing to poor outcomes. Additional factors contributing to cardiovascular outcomes include pulmonary hypertension; coexistent pulmonary, endocrine, and metabolic diseases; and risk factors for atherosclerotic disease.

Branch Pulmonary Artery Regurgitation in Repaired Tetralogy of Fallot: Correlation with Pulmonary Artery Morphology, Distensibility, and Right Ventricular Function.

Background: The aim was to determine the effect of pulmonary artery (PA) morphology on the branch pulmonary artery-regurgitation fraction (BPA-RF), the relationship of pulmonary insufficiency (PI) to BPA-RF and PA-distensibility, and factors (BPA-RF and PA-distensibility) associated with right ventricular function (RVF) in repaired tetralogy of Fallot (rTOF).

Methods: A total of 182 rTOF patients (median age 17.1 years) were analyzed for length, angle of PA, BPA-RF, PI, and PA-distensibility, using magnetic resonance imaging.

Ischemic stroke in Kawasaki disease.

Pediatric stroke is considered to be rare. Stroke resulting from cerebral vasculitis is also uncommon in young children. With the increasing prevalence of Kawasaki disease (KD) diagnosis, this acquired vasculitis has been reported with various clinical presentations including neurological symptoms.

Low cardiac output thyroid storm in a girl with Graves' disease.

A 15-year-old girl with Graves' disease presented with hypotension after methimazole and propranolol were re-started for hyperthyroidism. She was found to have pulmonary artery hypertension resulting in obstructive shock. Thyroid storm was diagnosed according to Burch and Wartofsky score.

5'UTR repeat polymorphisms of the BMPR2 gene in children with pulmonary hypertension associated with congenital heart disease.

Unlabelled: The mutations of bone morphogenetic protein receptor type 2 (BMPR2) in patients with idiopathic pulmonary hypertension has been well defined. We investigated the occurrence of BMPR2 mutation and genetic polymorphisms in children with pulmonary hypertension associated with congenital heart disease (aPH/CHD) and correlated with the pulmonary haemodynamic and vasoreactivity.

Methods: BMPR2 mutation/polymorphisms were determined in 30 aPH/CHD children.

Reversible course of pulmonary arterial hypertension related to bone marrow transplantation.

Pulmonary arterial hypertension (PAH) is considered to be a rare but serious complication of bone marrow transplantation (BMT). The majority of the reports demonstrated a potential fatal outcome, while treatments are postulated to require an indefinite duration. Our objective is to describe cases of reversible PAH related to BMT in two patients.

Transcoronary bone marrow-derived progenitor cells in a child with myocardial infarction: first pediatric experience.

Background: Recent advances in stem cell therapy to restore cardiac function have great promise for patients with congestive heart failure after myocardial infarction in an adult population.

Objective: We examined the benefits of bone marrow-derived progenitor cells treatment modality for the pediatric patient.

Methods And Results: We present our first case of transcoronary autologous stem cell transplantation in a 9-year-old girl with refractory congestive heart failure secondary to myocardial infarction 1 year after transcatheter revascularization.

Correlation between 6-min walk test and exercise stress test in healthy children.

Aim: To investigate the correlation between 6-min walk test (SMWT) and incremental treadmill exercise stress test (EST) as indicators for the functional capacity in children.

Methods: Healthy children aged 9-12 years were included. The anthropometric data, SMWT and EST were prospectively measured using the standard protocols.

Assessment of pulmonary vasoreactivity in children with pulmonary hypertension.

Purpose Of Review: With the current advance in understanding and treatment of pulmonary arterial hypertension in children, pulmonary vasoreactivity testing would navigate the treatment option. An inclusive review of the milestone studies and also recent literature over the last few years on the pulmonary vasoreactivity testing in children will provide the update on various available pulmonary vasodilator agents, markers related to vasoreactivity response, the implication of the testing result on child management and outlook for the long-term outcome.

Recent Findings: There continue to be emerging data regarding pulmonary vasodilators for vasoreactivity testing in children and the genetic predictor of pulmonary vasoreactivity response, particularly in children with idiopathic and familial pulmonary hypertension.

Comparison of vascular complications between conventional treatment and bone marrow transplantation for children with beta-thalassemia disease.

Patients with ss-thalassemia may be predisposed to premature atherosclerosis due to vascular dysfunction. This is observed in adults. Whether atherosclerosis changes in ss-thalassemia disease (BTD) occur early in childhood is not clear.

Aerosolized iloprost for pulmonary vasoreactivity testing in children with long-standing pulmonary hypertension related to congenital heart disease.

Background: In congenital heart disease with increased pulmonary blood flow and pressure, progressive changes in the vascular structure can lead to irreversible pulmonary hypertension (PH). Pulmonary hemodynamic parameters are used to determine whether surgical correction is no longer indicated. In this study, aerosolized iloprost was used to assess pulmonary vasoreactivity in children with long-standing PH related to congenital heart disease.

Aerosolized iloprost for postoperative pulmonary hypertensive crisis in children with congenital heart disease.

Introduction: Pulmonary hypertensive crisis (PHC) is a significant contributor to the morbidity and mortality of surgery for congenital heart defect. Management of such a potentially fatal complication has been evolving for the past decades. Inhaled iloprost has been reported as an alternative treatment for this condition.

Single-stage versus two-stage modified Fontan procedure.

We compared surgical outcomes of the single-stage and two-stage modified Fontan procedures to clarify clinical superiority. Of 28 children undergoing a modified Fontan procedure from October 1995 to October 2005, 15 had a 1-stage and 13 had a 2-stage operation. In the 2-stage group, pulmonary artery growth was evaluated before and after the first stage.

Left main coronary occlusion from Takayasu arteritis in an 8-year-old child.

Takayasu's arteritis (TA) is a chronic systemic inflammatory disease that usually affects the aorta and its primary branches and occasionally the coronary arteries. We report the case of an 8-year-old girl who was presented with chest pain and was referred to our institution with the presumptive diagnosis of pneumonia and sepsis. Ultimately, the patient's chest pain was attributed to myocardial infarction secondary to coronary occlusion from TA.

Pulmonary arterial hypertension after childhood cancer therapy and bone marrow transplantation.

According to the Third World Symposium on Pulmonary Arterial Hypertension (PAH), chemotherapy is considered to be one of the possible risk factors for patients developing PAH. However, to date, no literature has sufficiently addressed the risk, natural history, and effective treatment of this condition. We report our experience on how early diagnosis, detailed monitoring of disease course, and appropriate treatment application have led to a successful outcome of PAH management in childhood after cancer therapy.

Beraprost therapy in children with pulmonary hypertension secondary to congenital heart disease.

We present a report on children with severe pulmonary hypertension secondary to congenital heart disease who received 6 months of beraprost therapy. The children had an increase in intracardiac left-to-right shunt and a reduction of the pulmonary-to-systemic vascular resistance ratio, whereas the pulmonary artery pressure was not significantly changed.

Inhibition of K(+) channel activity in human pulmonary artery smooth muscle cells by serum from patients with pulmonary hypertension secondary to congenital heart disease.

Activity of K(+) channels regulates cytosolic free Ca(2+) concentration by controlling membrane potential. A rise in cytosolic free Ca(2+) concentration in pulmonary artery smooth muscle cells (PASMC) triggers pulmonary vasoconstriction and stimulates PASMC proliferation. Whether serum from children with pulmonary hypertension (PH) secondary to congenital cardiopulmonary diseases contains a factor(s) that inhibits K(+) channel function in PASMC was investigated using patch clamp techniques.

Upregulated TRP and enhanced capacitative Ca(2+) entry in human pulmonary artery myocytes during proliferation.

A rise in cytosolic Ca(2+) concentration ([Ca(2+)](cyt)) due to Ca(2+) release from intracellular Ca(2+) stores and Ca(2+) influx through plasmalemmal Ca(2+) channels plays a critical role in mitogen-mediated cell growth. Depletion of intracellular Ca(2+) stores triggers capacitative Ca(2+) entry (CCE), a mechanism involved in maintaining Ca(2+) influx and refilling intracellular Ca(2+) stores. Transient receptor potential (TRP) genes have been demonstrated to encode the store-operated Ca(2+) channels that are activated by Ca(2+) store depletion.

Sustained membrane depolarization and pulmonary artery smooth muscle cell proliferation.

Pulmonary vasoconstriction and vascular medial hypertrophy greatly contribute to the elevated pulmonary vascular resistance in patients with pulmonary hypertension. A rise in cytosolic free Ca(2+) ([Ca(2+)](cyt)) in pulmonary artery smooth muscle cells (PASMC) triggers vasoconstriction and stimulates cell growth. Membrane potential (E(m)) regulates [Ca(2+)](cyt) by governing Ca(2+) influx through voltage-dependent Ca(2+) channels.

Wire-snare technique with distal flow control for coil occlusion of a modified Blalock-Taussig shunt.

Coil occlusion of Blalock-Taussig shunts has been associated with a high rate of device embolization. We describe a technique consisting of transvenous snaring and exteriorization of a guidewire advanced through a modified left Blalock-Taussig shunt, allowing distal shunt flow control and successful coil occlusion of the shunt. Cathet.

Spontaneous resolution of myelofibrosis and pancytopenia followed by the development of acute myeloid leukemia with an extramedullary mass.

Purpose: Spontaneous resolution of myelofibrosis is extremely rare. A patient with myelofibrosis and pancytopenia that spontaneously resolved with subsequent development of acute myelomonocytic leukemia and an extramedullary mass of the ovary is described.

Patient And Methods: A 2-year-old girl had severe pancytopenia and myelofibrosis without signs of myeloid metaplasia.

Safety and immunogenicity of REMUNE in HIV-infected Thai subjects.

The safety and immunogenicity of REMUNE, an HIV-specific immune based therapy for HIV infection, was evaluated in a cohort of 30 HIV infected subjects in Thailand. This therapy utilizes a gp120 depleted inactivated virus (HZ321), which exhibits a high degree of conservation with the core antigens of both type B' and E strains of HIV, the predominant Thailand isolates. The treatment was well tolerated, with no serious adverse events reported over the course of the 4-month trial.

Factors affecting patients' acceptance of H.I.V. infections.

A study on patients' acceptance of the diagnosis of HIV infection was done in 36 patients at Ramathibodi Hospital. Patients were interviewed based on the data in a questionnaire. Over one half of the patients were 21-40 years old.