Utility of Echocardiography in Detecting Silent Complications After Pediatric Catheter Ablations.

Although transcatheter arrhythmia ablation (TCA) has been performed in children for over two decades, guidelines for routine use of post-ablation transthoracic echocardiography (TTE) are absent. We sought to determine the efficacy of TTE after apparently uneventful TCA procedures in detecting adverse findings and identify predisposing factors. A retrospective review of clinical and procedural data on patients who underwent TCA for supraventricular arrhythmias from 2000 to 2015 was performed. Pre- and post-ablation TTE data were reviewed. All patients were followed at 1 week, 6 and 12 months post-TCA. A repeat TTE was performed at 12 months on patients in whom post-TCA abnormalities were found. Patients were divided into two groups: those with and without adverse TTE findings and comparative analysis between variables was performed. Data on 252 patients, 52% males, mean age 14 ± 3 years were analyzed. New onset or worsening atrioventricular valve regurgitation occurred in 17 (6.7%), a small pericardial effusion in 3 (1.2%) and worsened ventricular function in 2 patients (0.8%). Patients in the complication group had higher mean number of ablations (22.6 ± 15.3 vs. 16.8 ± 9.2, p 0.001) and required longer duration of ablation (sec) (254.6 ± 256.4 vs. 180.9 ± 158.9, p < 0.001). TCA location (including coronary sinus), energy source, arrhythmia substrate, and a trans-septal approach were noncontributory to any adverse findings. Routine post-ablation TTE uncovers asymptomatic self-resolving abnormalities that typically do not require any intervention.

Association between anemia and packed cell transfusion and outcomes of ventricular septal defect and atrioventricular canal repair in children.

Data on the prevalence and impact of anemia and packed red blood cell (PRBC) transfusions in children with congenital heart disease are limited. Our objectives were to determine the prevalence of anemia and its impact and the impact of PRBC transfusion in the initial 5 days after surgical repair on postoperative outcomes in infants with ventricular septal defect (VSD) and atrioventricular (AV) canal. Retrospective chart review of infants (1 year old) (n = 195) with AV canal or VSD who underwent surgical repair at Children's Hospital of Michigan during a 10-year period. Statistical analyses (SPSS 17.0) included Chi square and Student t test as well as regression analysis with significance set at p = 0.05. Preoperative anemia was diagnosed in 45 of 195 (23%) children. Anemic infants had VSD more frequently (80%), significantly shorter bypass and cross-clamp durations, and higher red cell distribution widths. Postoperative outcomes and PRBC transfusions were similar in the groups. On regression analysis, AV canal was associated with a significantly lower (odds ratio 0.21; 95% confidence interval 0.07-0.68, p = 0.009) risk of anemia. Infants who received a PRBC transfusion (n = 42) had significantly lower birth weights as well as weights at surgery and longer postoperative durations of pressor use, ventilation, oxygen supplementation, and length of stay than those who did not (n = 153) receive transfusions. PRBC transfusion was independently associated with longer postoperative length of stay, oxygen, pressor use, and ventilator duration. Approximately 23% of infants with AV canal or VSD are anemic. PRBC transfusions in the initial 5 postoperative days, but not anemia, are independently associated with adverse postoperative outcomes. Further studies to evaluate conservative transfusion strategies in this population are needed.

Catheterization-based intervention in low birth weight infants less than 2.5 kg with acute and long-term outcome.

BACKGROUND: The number of low birth weight infants with congenital heart disease is increasing and catheterizations may have an increased risk for mortality and morbidity. OBJECTIVES: We investigate the outcome and complications of cardiac catheterizations in infants weighing < 2.5 kg. METHODS: Retrospective review of catheterization records from 1995 to 2010 in infants weighing < 2.5 kg. The demographics, procedure, outcome, and follow-up data were collected. RESULTS: Of 101 catheterizations performed in 88 patients, 45 (45%) catheterizations were interventional. Balloon atrial septostomy (n = 23), pulmonary valvuloplasty (14), aortic valvuloplasty (4), stent placement (3), balloon angioplasty (2), and temporary pacemaker insertion (1) were successfully performed. Balloon atrial septostomy was performed with pulmonary or aortic valvuloplasty in two catheterizations. Infants < 2.5 kg had higher significant adverse event rate that those 2.5-3.5 kg (13% versus 6.6%, P < 0.05). No procedural death was noted. Significant adverse events (n = 13) included cardiopulmonary resuscitation three, vascular six, arrhythmia three, and apnea requiring intubation one. On median follow-up of 3 years (0.03 to 14), n = 69, mortality rate was 36%. In six patients with valvar pulmonary stenosis with median follow-up of 6 years (0.75-13), four (67%) did not require re-intervention. Of two patients with aortic stenosis, one did not require repeat intervention for 6 years (last follow-up). CONCLUSION: Interventional catheterization is feasible with low procedural morbidity and mortality in high risk infants < 2.5 kg. Catheterization primarily serves as a palliative procedure to stabilize infants for definitive treatment. Balloon valvuloplasty may be effective for isolated valvar pulmonary stenosis in infants < 2.5 kg.

Persistent Pulmonary Hypertension of the Newborn with D-transposition of the Great Arteries: Management and Prognosis.

OBJECTIVE: There is a paucity of data on clinical correlates and outcomes of pulmonary hypertension (PH) in patients with D-transposition of the great arteries (D-TGA) in the era of inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO). Our objective was to compare clinical characteristics and outcomes of infants with D-TGA with and without PH, defined as hypoxemia that required iNO and/or ECMO. METHODS: We undertook a single-center retrospective chart review involving infants with gestational age ≥32 weeks with D-TGA who, underwent arterial switch operation over a 12-year period. Demographic and clinical data, details of the repair and postoperative complications were abstracted. RESULTS: Our cohort (n = 93), 61 (66%) of whom were males, had a mean (SD) gestational age and birth weight of 38.7 (1.8) weeks and 3.2 (0.6) kg, respectively. PH requiring iNO and/or ECMO was noted in 20 (21.5%) infants. Infants with PH had significantly lower birth weight [2.8 (0.56) vs. 3.33 (0.61)] and gestational age [37.7 (2.1) vs. 38.9 (1.7)] than those without PH. Rates of postoperative complications (duration of pressors, sedative medicaiton and duration of hospital stay, and mechanical ventilation were higher in the group with PH. Of the five (5.4%) infants who died, four received iNO and ECMO. Death or postoperative complications tended to be associated with lower gestational age [OR 0.689; 95% CI: 0.469-1.012, P = 0.058] but not with D-TGA category or bypass duration. CONCLUSIONS: Despite aggressive treatment with iNO and ECMO, the coexistence of PH in this population is associated with higher rates of mortality and postoperative complications. Our results also suggest that an early term birth may be associated with PH in infants with D-TGA.

Tetralogy of Fallot with complete DiGeorge syndrome: report of a case and a review of the literature.

Complete DiGeorge syndrome (CDGS) has a severe T-cell immunodeficiency and is fatal without thymus or bone marrow transplantation. Associated congenital heart disease (CHD) further complicates the clinical management. We report an infant with tetralogy of Fallot, confluent and hypoplastic pulmonary arteries, right aortic arch, and aberrant left subclavian artery. He was athymic with no CD3+ T cells. CDGS was diagnosed with 22q11.2 deletion. The patient underwent central aortopulmonary shunt at 12 days of age. The patient died at 5 weeks of age awaiting thymus transplantation. We performed a review of the literature regarding CDGS and CHD. We found 43 cases including conotruncal defects (20) and nonconotruncal defects (23). The overall mortality rate was 67%. Among 30 cases undergoing transplantation (bone marrow 16 and thymus 12, bone marrow + thymus 2), the mortality rate was 53%. The patients with conotruncal defects were more likely to die before transplantation (45% vs. 16%, P =.04). The main cause of death was infection before and after transplantation. We conclude that children with CDGS and CHD have a high mortality. Bone marrow and thymus transplantation can improve the survival, but the overall management of these high risk patients remains challenging.