Alisa Limsuwan

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. Methods: Children with long‐standing and severe PH secondary to congenital heart disease were included in this study. Various hemodynamic parameters were measured before and after iloprost inhalation (0.5 μg/kg), and vascular resistance was determined. Responders to the iloprost test were defined as those with a decrease in both pulmonary vascular resistance (PVR) and pulmonary‐to‐systemic vascular resistance ratio (Rp/Rs) of >10%. Results: Eighteen children aged between 7 months and 13 years with long‐standing and severe PH secondary to congenital heart disease were studied. Thirteen children had a positive response, resulting in a mean (± SD) decrease of PVR from 9.3 ± 4.6 to 4.6 ± 2.7 Wood U · m2 (P < 0.001), and a mean decrease of Rp/Rs from 0.54 ± 0.37 to 0.24 ± 0.14 (P = 0.005). Conclusions: Iloprost‐induced pulmonary vasodilator responses vary among children with PH related to congenital heart disease. The use of inhale iloprost in the cardiac catheterization laboratory results in pulmonary vasoreactivity for some of these children particularly a reduction in PVR and the pulmonary‐to‐systemic vascular resistance ratio.

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.

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. Our report reaffirmed the fact that PAH is now a recognized complication of chemotherapy and bone marrow transplantation for leukemia. Combined pulmonary vasodilator treatment has a beneficial effect in improving the patient’s condition and functional status as suggested by initial acute pulmonary vasodilator testing.

Transcoronary Bone Marrow-Derived Progenitor Cells in a Child With Myocardial Infarction: First Pediatric Experience

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.

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.

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 occulsion from TA. She underwent a successful stenting of the left main coronary artery with sirolimus-eluting stent. In a review of the literature, we discuss the demographic profile, clinical and radiographic findings, and available therapeutic options.

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. Despite a recent advance in pulmonary hypertension therapy leading to improved prognosis in children, the novel knowledge on standardized pulmonary vasoreactivity testing in children and its interpretation remain limited and controversial. Summary The precise definition of pulmonary vasoreactivity testing remains debatable, particularly in children with pulmonary hypertension related to congenital heart defect. Defining the responder, in order to navigate the treatment option, is frequently dictated by institutional experience and facilities. Meanwhile, the criteria for responder in children with idiopathic pulmonary artery hypertension are reasonably consistent. In general, responders seem to have less severe disease and better prognosis.

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. Operative mortality was 26.6% in the 1-stage group and 0% in the 2-stage group. The benefits of a previous bidirectional Glenn shunt were decreased cyanosis and volume overload, but there was no significant difference in pulmonary artery growth reflected in pulmonary artery indices before and after the bidirectional Glenn procedure. Older children underwent a 2-stage modified Fontan procedure and had better outcomes in terms of lower mortality, improved oxygen saturation, decreased volume load, and less deterioration of atrioventricular valve regurgitation.

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. She was promptly treated with anti‐thyroid drugs, inorganic iodide, corticosteroid, and respiratory support. Pulmonary hypertension was treated with inhaled nitric oxide until the clinical status improved. Propranolol was withdrawn due to poor cardiac function. We herein present a unique case of a difficult‐to‐treat Graves’ disease presenting with severe pulmonary hypertension resulting in low cardiac output thyroid storm.

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. Herein we describe the case of a KD patient presenting with stroke. A 15‐month‐old boy was referred due to stroke that occurred on the fifth day of febrile illness. He was initially admitted to another hospital due to fever and diarrhea. He was discharged and re‐admitted 2 days afterward due to left hemiplegia. During the 10 days of the second hospitalization, he had a presumptive diagnosis of encephalomeningitis. Upon referral to the present hospital, he was found to have right middle cerebral artery branch stenosis and fusiform aneurysms of the coronary arteries. Retrospectively, the patient had the full clinical criteria for KD diagnosis. Therefore, stroke could be considered as one of the uncommon clinical manifestations of KD.