World Journal for Pediatric and Congenital Heart Surgery最新文献

Background: There is currently no consensus regarding the use of surveillance cross-sectional imaging in pediatric patients after bidirectional cavopulmonary connection (BDCPC). We sought to determine how computed tomography with angiography (CTA) and cardiac magnetic resonance (CMR) imaging impacted the clinical management of pediatric patients after BDCPC.

Methods: A single-center retrospective study including patients with single ventricle who had BDCPC between 2010 and 2019, and CTA/CMR studies obtained in these patients, at ≤5 years of age, and with Glenn physiology. Repeat studies on the same patient were included if the clinical situation had changed. The impact of CTA/CMR studies was categorized as major, minor, or none.

Results: Twenty-four patients (63% male) and 30 imaging studies (22 CTAs) were included. 60% were obtained in patients with hypoplastic left heart syndrome (HLHS); most common indication was Follow-up after an intervention (23%). 6 CMRs were performed on stable HLHS patients as part of a research protocol, with no clinical concerns. The overall impact of CTA/CMR studies was major in 13 cases (43.3%). CTA/CMR studies performed ≥1 year of age (62.5% vs 21.4%, P = .02) and in non-HLHS patients (66.7% vs 27.8%, P = .035) were associated with major impact. Also, 2/6 Research studies were associated with a major impact.

Conclusions: CTA/CMR imaging in pediatric patients with SV after BDCPC was associated with significant clinical impact in over 40% of cases, with a higher impact if obtained in patients ≥1 year of age and in non-HLHS patients. We cannot disregard the possibility of CMR as a surveillance imaging modality in this population.

There has been a paradigm shift in the management of patients with congenital heart disease with a move away from conventional surgical treatment in favor of a percutaneous catheter-based approach across the spectrum of valvular heart diseases. The Sapien S3 valve implantation in the pulmonary position has been previously reported using a conventional transcatheter approach in patients with pulmonary insufficiency due to an enlarged right ventricular outflow tract. In this report, we present 2 unique cases of intraoperative hybrid implantation of Sapien S3 valves in patients with complex pulmonic and tricuspid valvular disease.

Objectives: Approximately 0.2% to 2.7% of children with congenital heart disease require a tracheostomy after cardiac surgery with the majority having single ventricle (SV) type heart lesions. Tracheostomy in SV patients is reported to be associated with high mortality. We hypothesized that short- and long-term survival of patients with SV heart disease would vary according to tracheostomy indication.

Methods: This is a single center, 20-year, retrospective review of all patients with SV heart disease who underwent tracheostomy. Demographic, cardiac anatomy, surgical, intensive care unit, and hospital course data were collected. The primary outcome was survival following tracheostomy. Secondary outcome was the completion of staged palliation to Fontan.

Results: In total, 25 patients with SV heart disease who underwent tracheostomy were included. Indications for tracheostomy included one or more of the following: tracheobronchomalacia (n = 8), vocal cord paralysis (n = 7), tracheal/subglottic stenosis (n = 6), primary respiratory insufficiency (n = 4), diaphragm paralysis (n = 3), suboptimal hemodynamics (n = 2), and other upper airway issues (n = 1). Survival at six months, one year, five years, and ten years was 76%, 68%, 63%, and 49%, respectively. Most patients completed Fontan palliation (64%). Patients who underwent tracheostomy for suboptimal hemodynamics and/or respiratory insufficiency had a higher mortality risk compared to those with indications of upper airway obstruction or diaphragm paralysis (hazard ratio 4.1, 95% confidence interval 1.2-13.7; P = .02).

Conclusions: Mortality risk varies according to tracheostomy indication in patients with SV heart disease. Tracheostomy may allow staged surgical palliation to proceed with acceptable risk if it was indicated for anatomic or functional airway dysfunction.

Presented is a case report of a 54-year-old male Jehovah's Witness diagnosed with an anomalous right coronary artery originating from the main pulmonary artery with the initial presentation with a history of episodic fatigue, chest pain, palpitation, and bodily weakness. The patient was managed with reimplantation of the anomalous coronary artery onto the ascending aorta.

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.

We present a case of a newborn with a rare presentation of obstructed supracardiac total anomalous pulmonary venous connection who required emergent cannulation to extracorporeal membrane oxygenation (ECMO). Computed tomographic angiography of the heart was performed and using novel virtual dissection techniques aided in surgical planning and guidance. Computed tomographic angiography can be successfully performed in neonates with complex congenital heart disease on ECMO without adjustment of flows to aid in surgical management and novel virtual dissection techniques aid in complex anatomical delineation and spatial orientation with noncardiac structures. The preoperative imaging in this case allowed for appropriate and detailed presurgical planning and contributed to the excellent outcome of this patient.

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.