Annals of Pediatric Cardiology最新文献

An anomalous right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital anomaly that does not have the typical presentation of the more common anomalous left coronary artery. We present an infant with tetralogy of Fallot with atypical findings on the preoperative echocardiogram. A cardiac computerized tomographic (CT) scan showed ARCAPA. This was confirmed intraoperatively and repaired successfully. Close attention to coronaries on echocardiography and a low threshold for additional imaging can successfully diagnose ARCAPA in the presence of additional congenital heart defects.

Objective: To study the applicability of on-table extubation (OTE) protocol following congenital cardiac surgery in a low-resource setting and its impact on the length of intensive care unit (ICU) stay, hospital stay, hospitalization cost, parental anxiety, and nurse anxiety.

Materials and methods: In this prospective, nonrandomized, observational single-center study, we included all children above 1 year of age undergoing congenital cardiac surgery. We evaluated them for the feasibility of OTE using a prespecified protocol following separation from cardiopulmonary bypass. The data were prospectively collected on 60 children more than 1 year of age, belonging to the Risk Adjustment for Congenital Heart Surgery 1, 2, 3, and 4 groups and divided into two groups: those who underwent successful OTE and those who were ventilated for any duration postoperatively (30 children in each group). Duration of hospital stay, ICU stay, and total hospital cost were collected. Anxiety levels of the primary caregiver (nurse) in the ICU and the mother were assessed immediately after the arrival of the child in the ICU using the State Trait Anxiety Inventory (STAI).

Results: Children who were extubated immediately following congenital cardiac surgery had significantly shorter ICU stay (median 20 [19, 22] h vs. 22 [20, 43] h [P < 0.05]). Patients extubated on table had a significant reduction in hospital cost {median Rs. 161,000 (138,330; 211,900), approximately USD 1970 (P < 0.05)} when compared to children who were ventilated postoperatively {median Rs. 201,422 (151,211; 211,900) , approximately USD 2464}. The anxiety level in mothers was significantly less when their child was extubated in the operating room (STAI 36.5 ± 5.4 vs. 47.4 ± 7.4, P < 0.001). However, for the same subset of patients, anxiety level was significantly higher in the ICU nurse (STAI 46.0 ± 5.6 vs. 37.8 ± 4.1, P < 0.05).

Conclusion: OTE following congenital cardiac surgery is associated with a shorter duration of ICU stay and hospital stay. It also reduces the total hospital cost and the anxiety level in mothers of children undergoing congenital heart surgery. However, the primary bedside caregiver during the child's ICU stay had increased anxiety managing patients with OTE.

A simplified technique of performing the extracardiac Fontan operation on cardiopulmonary bypass is described. The advantages of this technique are briefly discussed.

Berry syndrome is an extremely rare constellation of several congenital cardiac anomalies consisting of aortopulmonary window, aortic origin of the right pulmonary artery (AORPA), interrupted aortic arch or hypoplastic aortic arch or coarctation of the aorta, and an intact ventricular septum with high neonatal mortality rates. The disease is fatal with high mortality (90%) in the neonatal period with surviving patients mostly developing pulmonary hypertension. We describe the clinical presentation and diagnostic clues in two patients with Berry syndrome.

Katz-Wachtel phenomenon is an electrocardiogram (ECG) sign demonstrating large equiphasic precordial QRS complexes indicative of biventricular hypertrophy. This sign is most commonly seen in children with congenital heart disease and is rarely described in adults. We report a case where this ECG sign was noted in an adult with dilated cardiomyopathy and discuss the possible mechanisms for this unusual finding.

Tuberculous (TB) involvement of the vascular system has been reported in the preantibiotic era. We, hereby, report a case involving a teenage boy who presented to us with left upper limb pain followed by gradually progressive motor and sensory deficit over 1 month with preceding history of tuberculosis. Examination revealed a palpable, noncompressible, nonpulsatile swelling superior to the lateral third of the clavicle. Imaging through ultrasonography, computed tomography, and magnetic resonance imaging confirmed the presence of a pseudoaneurysm with compression of the underlying nerves. The child underwent surgical thrombectomy with pseudoaneurysm repair and arteriorrhaphy along with antitubercular medications with complete recovery at 6 months. The tissue staining, nucleic acid amplification tests, and histopathology confirmed TB etiology. Tuberculosis continues to remain a major health concern, especially in the developing world. High index of suspicion is necessary to diagnose such manifestations to avoid catastrophic sequelae.

Total cavopulmonary connection (Fontan) without using cardiopulmonary bypass (CPB) may be superior to Fontan on CPB. In the experience of many, a Fontan operation without CPB may be associated with a reduced need for inotropic support, improved outcomes in the postoperative phase including faster time to termination of mechanical ventilatory support, reduced intensive care unit stay, lower volume of pleural and peritoneal effusions, and decreased hospital stay, thereby rendering it more cost-effective. However, the operation is technically more difficult to perform than Fontan on CPB and requires significant modifications of surgical technique and alteration in overall management strategy. In this article, an alternative technique of performing total cavopulmonary connection without CPB is described. Its advantages are briefly discussed.