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.

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. The child received daily injections of granulocyte colony-stimulating factor for 3 days prior to the bone marrow aspiration. The bone marrow cells were isolated to constitute CD133+/CD34+ more than 90% of the total number. Subsequently, the progenitor cell suspension was injected via a transcoronary catheter without any complication. Three months after stem cell therapy, her cardiac function, assessed by both cardiac magnetic resonance and echocardiogram, has been improved with the left ventricular ejection fraction at 47% compared to the baseline of 30%. CONCLUSION: This is the first reported pediatric case of successful transcoronary injection of bone marrow-derived progenitor cells for end-stage heart disease. The procedure is considered safe and feasible for the pediatric population.

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.

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 microg/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 (R(p)/R(s)) 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 x m(2) (P < 0.001), and a mean decrease of R(p)/R(s) 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 inhaled 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.

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.

Pericardial effusion in primary systemic carnitine deficiency.

A patient with pericardial effusion and a complicated presentation of primary systemic carnitine deficiency (PSCD) is described. This is the first case of PSCD reported to have pericardial effusion. Compound heterozygosity for two mutations in the SLC22A5 gene, T440M and F23del, and four SLC22A5 polymorphisms (c.IVS3+6A>G, c.-77G>A, c.-78C>T, and p.S95S) were identified in the patient.

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.

Treatment of Kawasaki disease with a moderate dose (1 g/kg) of intravenous immunoglobulin.

BACKGROUND: Kawasaki disease is an acute febrile illness recognized most often in young children. Coronary abnormality is the most serious complication preventable with intravenous immunoglobulin (IVIG) administration. Various treatment regimens of IVIG have been reported. OBJECTIVE: To determine initial treatment failure and prevalence of coronary artery abnormality (CAA) in Kawasaki disease (KD) treated with a moderate dose (1 g/kg) of intravenous immunoglobulin (IVIG). METHOD: All patients with a diagnosis of KD who had initial treatment with 1 g/kg of IVIG at Ramathibodi Hospital between 1994 and 1998 were reviewed retrospectively. RESULTS: Thirty-one of 41(76%) patients responded completely to a single treatment with a moderate dose of IVIG (group A). The second dose of 1 g/kg of IVIG was required in 7 patients (17%) due to persistent fever more than 48 hours after the initial treatment (group B), and 3 patients (7%) required 3 doses of 1 g/kg of IVIG due to persistent fever after the second dose (group C). During the convalescent phase, there were 19 per cent, 29 per cent and 100 per cent of the patients in group A, B and C, respectively who developed CAA with an overall rate of 27 per cent. After 1-year follow-up, the prevalence of CAA had decreased to 3 per cent, 0 per cent and 67 per cent in the according groups with overall rate of 9.6 per cent. Only 1 patient in group C developed a giant aneurysm of the right coronary artery. CONCLUSION: The efficacy of a moderate dose (1 g/kg) of IVIG in preventing CAA is lower than that of the high dose regimen (2 g/kg) reported previously. Short duration of fever before starting IVIG and low hemoglobin level may be the risk factors of unresponsiveness to moderate-dose IVIG.

Oral ibuprofen and indomethacin for treatment of patent ductus arteriosus in premature infants: a randomized trial at Ramathibodi Hospital.

BACKGROUND: Ibuprofen given intravenously to premature newborn infants is a proven treatment for patent ductus arteriosus (PDA). The efficacy of ibuprofen is comparable to indomethacin in many clinical trials with fewer renal side effects. However, the intravenous form of ibuprofen is not available in Thailand, whereas, the oral suspension form is widely used for antipyretic treatment in children. Therefore, the authors investigated the possibilities of using oral ibuprofen for the treatment of PDA in premature newborn infants. OBJECTIVE: To assess whether oral ibuprofen at 10 mg/kg/dose daily for 3 days was as effective as indomethacin to treat symptomatic PDA in premature infants and to compare the side effects of oral ibuprofen to indomethacin. SUBJECTS AND METHOD: Eighteen premature infants with gestational ages less than 34 weeks born at Ramathibodi Hospital who developed symptomatic PDA were randomly assigned to receive three doses of either indomethacin (oral or intravenous administration 0.2 mg/kg/dose for three doses given at 12 hourly intervals or oral ibuprofen (10 mg/kg/dose for three doses given at 24 hourly intervals). The rates of ductal closure, infants' clinical courses, side effects and complications were recorded. RESULTS: Birth weight, gestational age, gender, age onset and number of infants who had respiratory distress syndrome were similar in both groups, PDA was closed in 7 of 9 infants given ibuprofen (78%) and in 8 of 9 infants given indomethacin (89%) (p > 0.05). The mean plasma concentration of ibuprofen was 28.05 microg/ml at 1 hour after the third dose. Neonates in the ibuprofen group had more urine output. However, the increment of serum BUN and creatinine were not significantly different in both groups. There were no significant differences in duration of ventilator support as well as number of patients with bronchopulmonary dysplasia, intraventricular hemorrhage, necrotizing enterocolitis and death in both groups. CONCLUSION: Oral ibuprofen therapy is as effective as indomethacin for the treatment of PDA in premature infants and seems to have fewer renal side effects.

The right and left ventricular function after surgical correction with pericardial monocusp in tetralogy of fallot: mid-term result.

BACKGROUND: Surgical repair of tetralogy of Fallot (TOF) with reconstruction of the right ventricular (RV) outflow tract invariably results in pulmonary regurgitation (PR). Chronic PR has been associated with RV dysfunction and ventricular arrhythmia. Pericardial monocusp has recently been used at Ramathibodi Hospital to preserve pulmonary valve function. OBJECTIVES: First, to study the competency of the pericardial monocusp, one-year after correction. Second, to assess the right and left ventricular (LV) functions after surgery. Third, to assess correlation between severity of PR and the characters of electrocardiography (ECG) and chest X-ray (CXR) after correction. METHOD: A cross-sectional study was conducted in patients who, had undergone total correction for TOF at least one year ago. The past medical history was retrospectively reviewed from the medical records. The patients who underwent surgical correction with and without pericardial monocusp were recruited into group I and group II, respectively. The clinical symptoms, QRS duration from ECG, and cardio-thoracic (CT) ratio from CXR were analyzed. From the echocardiographic standpoint, the LV systolic function was determined by LV fractional shortening (LVFS), whereas the RV systolic function was determined by the tricuspid annular plane systolic excursion (TAPSE). Restrictive physiology of the RV was determined by presence of antegrade flow across the pulmonary valve during diastole. RESULTS: Sixty four patients were enrolled in the study, 7 in group I and 57 in group II. The median follow-up time after the surgery was 6.5 years, which was 3 years in group I and 7 years in group II (p < 0.01). All patients in group I (100%) and 45 (80.4%) in group II had moderate or severe PR. The severity of PR, the RV and LV systolic functions were not statistically significantly different between the two groups (p > 0.01). The median of the LVFS was 32.4 per cent, and of the TAPSE was 10.5 mm. There was no restrictive physiology of the RV in all patients. There were no significant correlations between symptoms, CT-ratio, QRS duration and the severity of PR. CONCLUSIONS: The pericardial monocusp could neither reduce severity of PR nor improve right and left ventricular functions after 3 years follow-up post-operatively. However, the right and left ventricular performances in mid-term period remained insignificantly changed and severity of PR could not be predicted from symptoms and simple laboratory investigations.