Pongsak Khowsathit

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.

Cardiac CT angiography in children with congenital heart disease

Cardiac imaging plays an important role in both congenital and acquired heart diseases. Cardiac computed tomography (angiography) cCT(A) is a non-invasive, increasingly popular, complementary modality to echocardiography in evaluation of congenital heart diseases (CHD) in children. Despite radiation exposure, cCT(A) is now commonly used for evaluation of the complex CHD, giving information of both intra-cardiac and extra-cardiac anatomy, coronary arteries, and vascular structures. This review article will focus on the fundamentals and essentials for performing cCT(A) in children, including radiation dose awareness, basic techniques, and strengths and weaknesses of cCT(A) compared with cardiac magnetic resonance imaging (cMRI), and applications. The limitations of this modality will also be discussed, including the CHD for which cMRI may be substituted.

N-Terminal Pro-Brain Natriuretic Peptide as a Marker in Follow-Up Patients with Tetralogy of Fallot After Total Correction

Patients with tetralogy of Fallot (TOF) after total correction usually have residual pulmonary regurgitation resulting in right ventricular (RV) dilatation and dysfunction. This study was performed to evaluate N-terminal pro-brain natriuretic peptide (NT-proBNP) in predicting RV dilatation and RV dysfunction in TOF after total correction. Twenty-one patients with TOF after total correction (12 males and 9 females, 12.06 ± 2.54 years old) underwent echocardiography, cardiac magnetic resonance imaging (MRI), and blood sampling for NT-proBNP. Mean time after total correction was 7.59 ± 2.30 years. From cardiac MRI study, mean right ventricular end diastolic volume index (RVEDVi) was 148.36 ± 64.50 ml/m2 and mean right ventricular ejection fraction (RVEF) was 35.50 ± 10.50%. Right ventricular dilatation was considered if RVEDVi was >108 ml/m2 and RV dysfunction was considered if RVEF was <40%. A plasma NT-proBNP level of 115 pg/ml was identified by receiver operating characteristic analysis in predicting RV dilatation and/or dysfunction. At this value, the sensitivity and specificity for predicting RV dilatation, RV dysfunction, and both RV dilatation and dysfunction were 71 and 100%, 71 and 71%, and 83 and 78%, respectively. In conclusion, plasma NT-proBNP level may be helpful in follow-up patients. Plasma NT-proBNP levels >115 pg/ml can be used as a marker in the detection of RV dilatation and dysfunction.

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.

Pacing in preterm with hydrops fetalis due to congenital complete heart block.

Hydrops fetalis due to congenital complete heart block (CCHB) is a rare condition. The outcome of the preterm fetus with hydrops fetalis due to CCHB is poor, and is frequently associated with significant morbidity and mortality. The management of this condition is difficult. We report our experience in a hydropic preterm using staged pacing by applying left ventricular epicardial pacing with a temporary pacemaker and subsequently, left ventricular epicardial pacing with a permanent pacemaker.

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.

Echocardiographic Videodensity Index of the Coronary Arteries in Kawasaki Disease: A Predictor for Subsequent Coronary Artery Abnormality

Even IVIG was established as a standard of care for patients with Kawasaki disease, there are still significant numbers of patients who developed coronary artery abnormality (CAA) or were resistant to therapy. Inflammation at the walls of these arteries has been demonstrated earlier during the acute phase. Direct analysis on these sites may reflect the ongoing pathological process. The purpose of our study was to quantitatively analyze the videodensity (VD) of the coronary arterial walls indexed to that of myocardium (videodensity index-VDI). The VDI was evaluated as a potential predictor for subsequent CAA. Patients who fulfilled the diagnostic criteria, ranging in age from 2 months to 9 years, were studied. All the patients ware treated with IVIG 1-2 gm/kg. An HP Sonos 4500 echocardiographic system was used to acquire images of the coronary arteries. Optimal images of the major branches of the coronary arteries including the right ventricular free wall myocardium were digitally captured and analyzed for VD using the NIH Image software. The echocardiographic studies were performed at the time of diagnosis, 2 months, and 6 months later. Sixty-two of 72 patients (86%) had adequate echocardiographic images for analysis. Eleven patients developed small (6), medium (2), and giant (3) coronary artery aneurysms. The VDIs in acute phase ranged from 0.50 to 2.49. The ROC curve analysis using SPSS identified an optimal VDI cut-off at 1.32 with sensitivity of 82% and specificity of 78% for predicting the CAA. The mean interobserver variability of the measurement was 5%. In conclusion, echocardiographic videodensity index of the coronary arterial wall may predict for the subsequent occurrence of coronary artery abnormality in patients with Kawasaki disease.Even IVIG was established as a standard of care for patients with Kawasaki disease, there are still significant numbers of patients who developed coronary artery abnormality (CAA) or were resistant to therapy. Inflammation at the walls of these arteries has been demonstrated earlier during the acute phase. Direct analysis on these sites may reflect the ongoing pathological process. The purpose of our study was to quantitatively analyze the videodensity (VD) of the coronary arterial walls indexed to that of myocardium (videodensity index-VDI). The VDI was evaluated as a potential predictor for subsequent CAA. Patients who fulfilled the diagnostic criteria, ranging in age from 2 months to 9 years, were studied. All the patients ware treated with IVIG 1-2 gm/kg. An HP Sonos 4500 echocardiographic system was used to acquire images of the coronary arteries. Optimal images of the major branches of the coronary arteries including the right ventricular free wall myocardium were digitally captured and analyzed for VD using the NIH Image software. The echocardiographic studies were performed at the time of diagnosis, 2 months, and 6 months later. Sixty-two of 72 patients (86%) had adequate echocardiographic images for analysis. Eleven patients developed small (6), medium (2), and giant (3) coronary artery aneurysms. The VDIs in acute phase ranged from 0.50 to 2.49. The ROC curve analysis using SPSS identified an optimal VDI cut-off at 1.32 with sensitivity of 82% and specificity of 78% for predicting the CAA. The mean interobserver variability of the measurement was 5%. In conclusion, echocardiographic videodensity index of the coronary arterial wall may predict for the subsequent occurrence of coronary artery abnormality in patients with Kawasaki disease.

Correction of absent pulmonary valve syndrome using a pericardial valved conduit.

Absent pulmonary valve syndrome in a 4-month-old infant was successfully corrected using a fresh autologous pericardial trileaflet valved conduit. He recovered from operation with only mild pulmonary regurgitation at 4 months postoperatively. This technique is an effective alternative for infants with congenital heart disease who need tissue valved conduits. It may be more suitable than the aortic homograft by reason of the shortage of small homografts and its lower costs.