Pulmonary arteriovenous malformations in children after the Kawashima procedure: Risk factors and midterm outcome.

BACKGROUND: Pulmonary arteriovenous malformations (PAVMs) are the major cause of progressive cyanosis in patients palliated with bidirectional cavopulmonary connection (BCPC). The aim of our study is to analyze the occurrence of PAVMs in patients after Kawashima procedure, to study the effect of total cavopulmonary connection (TCPC) on PAVMs, to evaluate the effect of axillary arteriovenous fistula (AAVF) creation on PAVMs, and to study the risk factors for PAVMs. METHODS: In this retrospective cohort study, all patients with left isomerism and azygous continuation of an interrupted inferior vena cava who underwent Kawashima procedure from July 2001 to December 2017 were included. RESULTS: Twenty.six patients after Kawashima procedure were included in our study. PAVMs were diagnosed in 12 patients (46%). Five of these 12 patients underwent TCPC with complete resolution of hypoxemia. Three patients underwent AAVF creation, 2 had complete resolution, while 1 had partial resolution of hypoxemia. Fourteen patients (54%) did not develop PAVMs. Nakata index below 267 mm2/m2and McGoon ratio below 1.9 predicted the development of PAVMs with high sensitivity and specificity. CONCLUSIONS: PAVMs represent a serious complication in patients who undergo Kawashima procedure. Small size of pulmonary arteries is an important risk factor for the development of PAVMs. Resolution of hypoxemia after TCPC completion supports the hepatic factor hypothesis. Early TCPC completion in these patients may help to avoid the development of PAVMs by restoring the hepatic factor. Resolution of hypoxemia after AAVF creation may support the lack of pulsatile flow hypothesis.

Late-presenting complete heart block after pediatric cardiac surgery.

Late presenting complete heart block after pediatric cardiac surgery is a rare complication and its management is well defined once the initial diagnosis in made timely and appropriately. In this report we described a child who underwent atrioventricular septal defect repair with a normal sinus rhythm during the postoperative period, as well as during the first 2 years of follow up. She subsequently developed complete heart block with bradycardia that required insertion of a pacemaker. Here we discuss this unusual late-presenting complication, possible risk factors, and management.

Ebstein cardiac anomaly, functional pulmonary atresia and isovaleric acidemia: A case report.

In this report, we present a rare association between Ebstein anomaly (EA) and isovaleric acidemia (IVA) in a newborn who was admitted to our cardiac center. He underwent for PDA stenting to maintain adequate pulmonary blood flow, later he developed recurrent metabolic acidosis, prominent sweaty feet odor, neutropenia and thrombocytopenia. His organic acids profile in the urine confirmed the diagnosis of IVA. To the best of our knowledge, there is no association between these two rare diseases. We are presenting this case report to highlight this rare association.

Outcome of tracheostomy after pediatric cardiac surgery.

OBJECTIVE: To investigate the incidence, timing indications and outcome of tracheotomy in children who underwent cardiac surgeries. METHODS: All pediatric cardiac patients (under 14 years of age) who underwent cardiac surgeries and required tracheotomy from November 2000 to November 2010 were reviewed. The data were collected and reviewed retrospectively. RESULTS: Sixteen children underwent tracheotomy after cardiac surgery. Fifteen of these cases had surgery for congenital heart disease, and one had surgery for an acquired rheumatic mitral valve disease. The mean ± SEMs of the durations of ventilation before and after tracheotomy were 60.4 ± 9.8 and 14.5 ± 4.79 days respectively (P value 0.0002). The means ± SEM of the lengths of ICU stay before and after tracheotomy were 63.31 ± 10.15 and 22 ± 5.4 days respectively (P value 0.0012). After the tracheotomy 12/16 patients (75%) were weaned from their ventilators and 10/16 were discharged from the PCICU. Six patients were discharged from the hospital and 3 were successfully decannulated. The overall survival rate was 9/16 (56%). CONCLUSION: Tracheostomy shortens the duration of mechanical ventilation and facilitates discharge from the ICU. The mortality of tracheotomy patients is still significant but is mainly related to the primary cardiac disease.