World journal for pediatric & congenital heart surgery最新文献

Purpose: Permanent pacing in the young pediatric population can be challenging, especially in small neonates. We describe our institutional experience with the surgical placement of dual-chamber permanent pacemaker (PPM) systems in neonates weighing <3 kg at the time of implantation. Methods: The Cincinnati Children's Hospital database was used to identify patients who had an epicardial dual-chamber PPM and weighed <3 kg at implantation from 2000 to 2022. Descriptive analyses focused on surgical techniques, postimplant complications, and survival. Results: Twenty patients underwent dual-chamber PPM implantation. Median age at implantation was 6.0 days [3.0-27.0], weight 2.6 kg [2.2-3.0], with the smallest being 1.9 kg. Eight of 20 patients (40%) were premature (<37 weeks), and 2/20 (10%) had Trisomy 21. Most implantations were via median sternotomy [17/20 (85%)]. Most had generator placement in the left upper quadrant [13/20 (65%)] above the posterior fascia and below the rectus muscle. Surgical indication for implantation was congenital heart block [18/20 (90%)], of which 4/18 (22%) were due to maternal lupus. Two patients had PPM secondary to postoperative heart block and 3/20 (15%) had emergent temporary epicardial wires placed before PPM. Pacemaker-related complications occurred in 1/20 (5%) patient on postoperative day 20 related to minor wound dehiscence, without infection, leading to a dual-chamber PPM exchange for a single-chamber device. Another patient (5%) developed a seroma requiring drainage on postoperative day 16. There were no PPM-related in-hospital mortalities or other complications, including late PPM infections. Conclusions: Dual-chamber epicardial PM implantation in neonates <3 kg is feasible with minimal complications and good outcomes based on our reported technique.

BackgroundDelayed sternal closure (DSC) is a well-established management strategy following complex congenital cardiac surgery that is used to mitigate postoperative hemodynamic and respiratory instability. It is mostly used in neonates requiring prolonged cardiopulmonary bypass (CPB), long aortic cross-clamp times, or deep hypothermic circulatory arrest who are predisposed to myocardial edema or bleeding. Our study evaluates morbidity and mortality after DSC in neonates including superficial and deep sternal wound infections, along with requirement for surgical debridement.MethodsRetrospective review of neonates who underwent DSC after cardiac surgery at a single center from 2015 to 2021.ResultsA total of 187 neonates were identified. Mean age and weight were 12.8 ± 6.8 days and 3.3 ± 0.5 kg, respectively. Mean days of open chest were 3.8 ± 5.8 days. Two neonates (1.07%) required sternal wound debridement, while 19 cases (10.2%) had superficial wound infections. Mean intensive care unit (ICU) and hospital stay were 12.8 ± 16.6 and 25.9 ± 36.9 days, respectively; 30-day mortality occurred in nine of 187 cases (4.8%). Univariate analysis indicated that DSC days (P = .01), extracorporeal membrane oxygenation (ECMO) (P = .000), aortic cross clamp time (P = .007), and CPB time (P = .006) were associated with 30-day mortality, while in multivariable analysis, only ECMO was significant (P = .002). Risk Adjustment for Congenital Heart Surgery (RACHS-1) score was the only independent risk factor for sternal wound infection in univariate analysis (P = .019) and multivariable analysis (P = .05).ConclusionDelayed sternal closure is a safe therapeutic option following complex neonatal cardiac surgery, where cardiac compression by sternal approximation is not tolerated due to myocardial edema, hemodynamic instability, or coagulopathy. Higher RACHS-1 score was associated with a greater incidence of sternal wound infections.

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is an extremely rare congenital coronary artery anomaly. Diagnosis is often made incidentally when evaluating for a murmur or other congenital heart disease. The ARCAPA may present with right heart failure and/or sudden cardiac death and surgical repair is recommended once the diagnosis is made.A seven-year-old child previously diagnosed with small ventricular septal defects presented to our cardiology clinic as a new patient. Echocardiogram revealed suspicion of a coronary anomaly and computed tomography angiography (CTA) confirmed a diagnosis of ARCAPA. She underwent successful surgical reimplantation of the anomalous right coronary artery and made an excellent recovery.This case highlights the importance of maintaining a high index of suspicion for coronary anomalies and strongly considering CTA in patients with imaging findings or symptoms consistent with ARCAPA.

Severe tricuspid regurgitation (TR) can lead to significant enlargement of the right atrium (RA) and poses unique clinical challenges. We report this case of a 17-year-old boy previously misdiagnosed with Ebstein anomaly who presented with dyspnea and palpitations. Initial examination revealed irregular heart rhythm, distended neck veins, and a significant murmur. Echocardiogram and computed tomography showed severe TR due to noncoaptation of the tricuspid valve, causing massive RA enlargement (1916 mL). The patient underwent successful surgical intervention, including tricuspid valve replacement and RA reduction. Postoperative complications included atrial fibrillation, which was medically managed. To our knowledge, this case had the largest RA to be reported and surgically treated in the literature. The case underscores the diagnostic challenges in differentiating severe TR from Ebstein anomaly and the progression from TR to significant RA enlargement. Accurate diagnosis and timely surgical management are critical in such cases to achieve favorable outcomes.

We present a patient who as an infant required mitral and tricuspid valve replacement, with a mechanical valve used in the mitral position and a surgeon fashioned extracellular matrix of porcine small intestinal submucosa (SIS-ECM) (CorMatrix) valve in the tricuspid position. Five years later, the mechanical valve required replacement with a larger mechanical valve. Although the CorMatrix tricuspid valve did not show significant signs of thrombosis or calcification, it was also replaced with a mechanical valve. That mechanical valve thrombosed twice within one year and both times required surgery for replacement. After the second thrombotic event, however, a new hand sutured SIS-ECM valve was used in place of a mechanical valve. The new SIS-ECM tricuspid valve continues to function appropriately 17 months later, and the patient has remained asymptomatic.

There is a paucity of data available regarding operative timing and approach for d-loop transposition of the great arteries (dTGA) with intact ventricular septum (IVS) in premature infants. We reviewed our surgical experience in a case series of five premature infants (<37 weeks gestational age) with dTGA/IVS and birthweights <2.0 kg. All five infants underwent primary arterial switch operation (ASO) at a median postnatal age of 19 days and weight of 1.8 kg. None of the infants had major postsurgical complications or needed reoperation. All infants were discharged home on room air. We demonstrated that premature infants <2.0 kg with dTGA/IVS can successfully undergo the ASO at <3 weeks postnatal age with no significant cardiac or neonatal complications.

Background: Neonatal management of congenital heart defects (CHD) among heterotaxy patients remains challenging due to significant heterogeneity in cardiac and visceral phenotypes. This study evaluated contemporary surgical outcomes and identified high-risk anatomic substrates. Methods: A total of 41 heterotaxy CHD patients who underwent neonatal surgical repair and/or palliation over a 10-year period were evaluated at a single institution. Heterotaxy anatomy was characterized according to right atrial isomerism (RAI) or left atrial isomerism (LAI), and other cardiac defects. Multivariate Cox regression and Kaplan-Meier analyses evaluated operative and intermediate-term outcomes. Results: Median age at initial operation was 7.0 days, and median operative weight was 3.1 kg. Median follow-up was 2.3 years. Of the total 41 patients, 27 (66%) had RAI, and 14 (34%) had LAI. Functional single ventricle anatomy was present in 30/41 patients (73%); 28/30 (93.3%) patients achieved stage II bidirectional Glenn and 14/30 (46.7%) achieved stage III Fontan completion; 1/30 (2%) patient underwent biventricular conversion. Operative mortality was 9.8% (4/41) after the initial operation. Permanent pacemaker placement was more common in patients with LAI. Interstage death rate was 10%, with no deaths after Fontan. Obstructed total anomalous pulmonary venous connection (TAPVC) was a risk factor for overall mortality (hazard ratio [6.0]; 95% confidence interval, 1.2-31.2; P = .033). Kaplan-Meier survival analysis demonstrated decreased five-year survival in RAI patients, LAI patients with single ventricle physiology, and RAI patients with obstructed TAPVC. Conclusions: Cardiac surgical outcomes for neonates with heterotaxy syndrome have significantly improved. Right atrial isomerism anatomy, single ventricle LAI patients, and RAI patients with obstructed TAPVC are associated with increased operative risk.

Objective: This study aimed to determine the effect of the degree of infundibular stenosis on growth of the pulmonary valve (PV) annulus in patients with tetralogy of Fallot (ToF). Methods: We reviewed clinical data of all patients who underwent ToF repair between 2002 and 2022. The diameter of the infundibular area was measured before ToF repair using a lateral view of angiography at end-systole and divided by the body surface area (infundibular index [mm/m²]). The cohort was divided into two groups, a systemic-to-pulmonary artery shunt (SPS) and a primary intracardiac repair (ICR), and compared for pulmonary annular growth and the infundibular index. Results: A total of 119 patients were included; the SPS group (n = 54) and the primary ICR group (n = 65). At ToF repair, the PV annulus was preserved in 43/65 patients (66%) in the primary ICR group and 23/54 (43%) in the SPS group (P = .0098). In a linear regression analysis, the infundibular index was significantly associated with PV growth (P < .001). The growth of the PV annulus was significantly associated with the infundibular index in both groups. In the 60 patients with a small PV annulus (Z-score < -2.0) at the initial presentation, logistic regression analysis showed that the infundibular index was the only factor significantly associated with PV preservation at ToF repair (P = .005). Conclusion: The degree of infundibular stenosis has a significantly greater effect on pulmonary annular growth than the presence of an SPS, which may affect the preservation of the PV at the time of ToF repair.

ObjectivesReview our clinical experience with eight patients at the University of Florida undergoing En-bloc combined heart+liver transplantation (ECH + LTX).MethodsContinuous variables are reported as median (interquartile range = IQR) and categorical variables are reported as N (%).ResultsEight patients underwent ECH + LTX between August 2020 and May 2023 at the University of Florida, with triple heart+liver+kidney transplantation performed in 2/8 = 25%. Median age at ECH + LTX was 47.34 years (IQR = 33.66-53.37), and all eight patients were >18 years of age. Six out of eight patients (75%) had congenital heart disease (CHD): one had biventricular CHD and five had functionally univentricular circulation and Fontan failure. Two out of eight patients (25%) had structurally normal hearts and acquired heart disease: one patient with hemochromatosis and combined cardiac and hepatic failure with nonischemic restrictive cardiomyopathy and one patient with nonischemic cardiomyopathy and alcoholic cirrhosis. Median wait list time was 93 days (IQR = 27.50-176.25). Three patients (3/8 = 37.5%) were supported with an intra-aortic balloon pump prior to ECH + LTX, and two of these three patients were subsequently also supported with extracorporeal membrane oxygenation secondary to progressive decompensation prior to ECH + LTX. Median hospital length of stay was 147 days. Median posttransplant length of stay was of 29 days. Seven of eight patients survived ECH + LTX and are alive today. One patient died two days after ECH + LTX. Mean length of follow-up after ECH + LTX of seven surviving patients (years) is 3.60 ± 0.38 (median = 3.79, IQR = 3.05-4.38, range = 1.91-4.64).ConclusionEn-bloc heart-liver transplantation is an effective treatment option for patients with combined heart and liver failure.