World Journal for Pediatric and Congenital Heart Surgery最新文献

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital malformation, usually diagnosed in the infant period due to myocardial ischemia and heart failure, with the need for emergency surgery. Less commonly, it can be asymptomatic until adulthood. Coronary artery aneurysms are also rare anatomical anomalies with symptoms of acute or chronic angina or even remain completely asymptomatic. We present an unusual case of ALCAPA, associated with a giant aneurysm of the right coronary artery. Meeting presentation: American Association for Thoracic Surgery 102nd annual meeting, Boston MA, USA, May 16, 2022.

Objectives: We reviewed outcomes in all 36 consecutive children <5 kg supported with the Berlin Heart pulsatile ventricular assist device (VAD) at the University of Florida, comparing those with univentricular circulation (n  =  23) to those with biventricular circulation (n  =  13).

Methods: The primary outcome was mortality. Kaplan-Meier methods and log-rank tests were used to assess group differences in long-term survival after VAD insertion. T-tests using estimated survival proportions and standard errors were used to compare groups at specific time points.

Results: Of all 82 patients ever supported with Berlin Heart at our institution, 49 (49/82  =  59.76%) weighed <10 kg and 36 (36/82  =  43.90%) weighed <5 kg. Of these 36 patients who weighed <5 kg, 26 (26/36  =  72.22%) were successfully bridged to transplantation. Of these 36 patients who weighed <5 kg, 13 (13/36  =  36.1%) had biventricular circulation and were supported with 12 biventricular assist devices (BiVADs) and 1 left ventricular assist device (LVAD) (Age [days]: median  =  67, range  =  17-212; Weight [kilograms]: median  =  4.1, range  =  3.1-4.9), while 23 (23/36  =  63.9%) had univentricular circulation and were supported with 23 single ventricle-ventricular assist devices (sVADs) (Age [days]: median  =  25, range  =  4-215; Weight [kilograms]: median  =  3.4, range  =  2.4-4.9). Of 13 biventricular patients who weighed <5 kg, 12 (12/23  =  92.3%) were successfully bridged to cardiac transplantation. Of 23 functionally univentricular patients who weighed <5 kg, 14 (14/23  =  60.87%) were successfully bridged to cardiac transplantation. For all 36 patients who weighed <5 kg: 1-year survival estimate after VAD insertion  =  62.7% (95% confidence interval [CI]  =  48.5%-81.2%) and 5-year survival estimate after VAD insertion  =  58.5% (95% CI  =  43.8%-78.3%). One-year survival after VAD insertion: 84.6% (95% CI  =  67.1%-99.9%) in biventricular patients and 49.7% (95% CI  =  32.3%-76.4%) in univentricular patients, P  =  0.018. Three-year survival after VAD insertion: 84.6% (95% CI  =  67.1%-99.9%) in biventricular patients and 41.4% (95% CI  =  23.6%-72.5%) in univentricular patients, P  =  0.005.

Conclusion: Pulsatile VAD facilitates bridge to transplantation in neonates and infants weighing <5 kg; however, survival after VAD insertion in these small patients is less in those with univentricular circulation in comparison to those with biventricular circulation.

Background: Regionalization of care for children with congenital heart disease has been proposed as a method to improve outcomes. This has raised concerns about limiting access to care. We present the details of a joint pediatric heart care program (JPHCP) which utilized regionalization and actually improved access to care. Methods: In 2017, Kentucky Children's Hospital (KCH) launched the JPHCP with Cincinnati Children's Hospital Medical Center (CCHMC). This unique satellite model was the product of several years of planning, leading to a comprehensive strategy with shared personnel, conferences, and a robust transfer system; "one program-two sites." Results: Between March 2017 and the end of June 2022, 355 operations were performed at KCH under the auspices of the JPHCP. As of the most recent published Society of Thoracic Surgeons (STS) outcome report (through the end of June 2021), for all STAT categories, the JPHCP at KCH outperformed the STS overall in postoperative length of stay, and the mortality rate was lower than expected for the case mix. Of the 355 operations, there were 131 STAT 1, 148 STAT 2, 40 STAT 3, and 36 STAT 4 operations, with two operative mortalities: an adult undergoing surgery for Ebstein anomaly, and a premature infant who died from severe lung disease many months after aortopexy. Conclusions: With a select case mix, and by affiliating with a large volume congenital heart center, the creation of the JPHCP at KCH was able to achieve excellent congenital heart surgery results. Importantly, access to care was improved for those children at the more remote location utilizing this one program-two sites model.

An 18-year-old male with complex single-ventricle physiology status post-3-stage palliation developed a large Norwood aneurysm (77 × 67 mm). The patient underwent a successful surgical reconstruction. Care providers must be aware of this rare complication and provide appropriate surveillance.

A systematic review was performed for evaluation of the performance of CardioCel^® in cardiac surgery. The review included all studies published from January 2013 to December 2020. We conclude that CardioCel is a strong, flexible tissue substitute with good handling characteristics and a low incidence of thrombosis, aneurysm formation, infection, or structural degeneration. It can be used for a variety of intracardiac and extracardiac repairs of congenital heart defects in all age groups with good durability at mid-term follow-up. However, the use of CardioCel in certain positions requires caution. Information on the long-term performance of CardioCel is lacking.

The congenital heart surgeon frequently encounters patients with various genetic disorders requiring surgical intervention. Although the specifics of the genetics for these patients and their families lie in the purview of specialists in genetics, the surgeon is well-served to be familiar with aspects of specific syndromes that impact surgical management and perioperative care. This aids in counseling families in expectations for the hospital course and recovery as well as can impact intraoperative and surgical management. This review article summarizes key characteristics for the congenital heart surgeon to be familiar with for common genetic disorders as they help coordinate care.

The aim of this study is to demonstrate the safety and advantages of a multidisciplinary approach to surgical resection of mediastinal masses in children. Eight patients underwent resection of a mediastinal mass by a team involving both a pediatric general surgeon and pediatric cardiothoracic surgeon. One patient required rapid initiation of cardiopulmonary bypass to complete the tumor resection and repair an aortic injury that occurred when removing adherent tumor from the structure. Perioperative outcomes were excellent for all patients. This series shows that a multidisciplinary surgical approach can be potentially life saving.

Interrupted aortic arch is a rare congenital heart defect. Among patients with interrupted aortic arch, an interruption between the innominate artery and left common carotid artery (type C) is the rarest. Herein, we report the case of a neonate with aortic atresia, ventricular septal defect, type C interrupted aortic arch, and aberrant right subclavian artery who underwent Norwood stage I operation and staged biventricular repair. Due to aortic atresia, coronary artery perfusion was retrograde from the right common carotid artery. The surgical technique and special considerations for cardiopulmonary bypass are presented.