World journal for pediatric & congenital heart surgery最新文献

Background: With an increasing number of Fontan patients surviving into adulthood, the burden of end-stage heart failure is increasing. Prior studies have reported suboptimal heart transplantation (HTx) outcomes. Therefore, the authors describe their institutional experience of HTx in patients with Fontan circulation failure, including en-bloc heart-liver transplantation (HLTx) and pretransplant systemic ventricular assist device (SVAD) therapies.

Methods: All consecutive Fontan-palliated patients undergoing HTx or HLTx between 2013 and 2022 at the authors' institution were included. Pretransplant characteristics and posttransplant outcomes were recorded.

Results: Twenty-six transplant recipients, including 5 (19%) HLTx recipients, were identified. The majority (n = 16, 62%) of patients had a cardiac diagnosis of hypoplastic left heart syndrome. Seven (26%) patients were bridged to transplant on an SVAD; the median duration of support was 104 [IQR 39-543] days. Transplantation occurred at a median of 10.6 [IQR 6.8-15.6] years post-Fontan. Fourteen (54%) patients had ≥1 in-hospital complication and 4 (15%) patients required an in-hospital reoperation. Postdischarge reinterventions included tricuspid valve repair (n = 1, 4%) and retransplantation (n = 1, 4%). Two (8%) in-hospital mortalities [22 days (post-HLTx), 2.9 months (post-HTx] and 4 (15%) postdischarge mortalities [5.4 months, 3.1 years, 5.7 years, 7.1 years (all post-HTx)] occurred. Overall survival was excellent, with in-hospital, one-year, and five-year actuarial survival being 92%, 89%, and 80%, respectively.

Conclusions: The current series demonstrates that excellent posttransplant outcomes are achievable for patients with Fontan circulation failure using a comprehensive approach including SVAD and HLTx therapies. Pretransplant optimization, sometimes including SVAD implantation, and meticulous operative planning are imperative strategies for successful patient outcomes.

Cardiac disease in young children can be unrecognized until symptoms are unmasked by a precipitating event, such as an infection. We present a case of anomalous left coronary artery from the pulmonary artery causing clinically significant disease in a four-month-old male with concomitant mitral regurgitation and pulmonary coccidioidomycosis who required modification of his surgical management due to the infection. This case highlights how timely diagnosis and perioperative management and recovery can be affected by concurrent infections in patients with congenital heart disease.

The presentation of pulmonary vasculature in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) is highly variable-as is the number, size and position of the MAPCAs and their relationship with the native pulmonary artery system. The priority in the management of this disease should be attaining timely and complete unifocalization, as opposed to single-stage full repair in every case. The merit of early unifocalization is that it secures the pulmonary vascular bed by (a) avoiding loss of lung segments from progressive stenosis/atresia of MAPCA origins, (b) preventing lung injury from high pressure/flow in areas fed by large, unobstructed MAPCAs, and (c) restoring central continuity of the pulmonary vasculature. Furthermore, there are a small but important group of patients with poorly developed vessels (about 10%-15% of the population) and/or diminutive native pulmonary artery vasculature that require initial shunt procedures to promote rehabilitation and growth of vessels before unifocalization can be attempted. During unifocalization, patients not suitable for single stage repair can be identified by intraoperative flow studies and can be successfully managed with staged strategies that provide time for growth and reinterventions on the pulmonary vasculature. Over 85% of patients can achieve unifocalization. Deferring closure of the VSD to a subsequent procedure is safe and these cases have similar survival to primary repair. Some patients (15%-20%) may never achieve VSD closure with this strategy but can still maintain a good quality of life with a restrictive right ventricular to pulmonary artery conduit and open VSD.

Pulmonary atresia with ventricular septal defect (PA-VSD) is usually diagnosed by transthoracic or fetal echocardiography, with the prenatal diagnosis being feasible and accurate if fetal cardiology services are available. The limitations of transthoracic echocardiography (TTE) in the evaluation of PA-VSD include the complete evaluation of the pulmonary arteries and patent ductus arteriosus, quantitative evaluation of the right ventricle size and function, and delineation of associated cardiac anomalies such as coronary artery anomalies, anomalies of systemic or pulmonary venous return, and complex arch anomalies. Echocardiography also has limitations in evaluating hemodynamics such as flow volumes, shunts, and regurgitant fraction. Despite these limitations, TTE remains the most widely available and the most cost-effective cardiac imaging modality for patients with PA-VSD and its accuracy in evaluating the sources of pulmonary blood flow and in selecting patients for systemic-to-pulmonary artery shunt palliation is well established. Cardiac computed tomography (CT) can answer many of the questions not answered by TTE, including demonstrating the PA anatomy and defining coronary artery, systemic and pulmonary venous, and aortic arch anatomies. The short acquisition time allows for the study to be performed without sedation/anesthesia in most patients. Cardiac CT is also useful in defining postoperative anatomy when there are non-magnetic resonance imaging (MRI) compatible devices or even MRI-compatible devices that cause a significant MRI artifact. Cardiac MRI/magnetic resonance angiography has emerged as an ideal modality to evaluate patients with PA-VSD as it allows for anatomic, functional, and hemodynamic assessment without exposure to ionizing radiation or iodinated contrast material. Both cardiac CT and cardiac MRI can be used to generate 3D imaging of the heart.

We present a case of severe hypertriglyceridemia (HTG) in a 21-month old female undergoing cardiac surgery for a ventricular septal defect and subaortic membrane excision. During the operation, a milky white supernatant was observed in the cardiopulmonary bypass circuit, prompting immediate lipid profile testing that revealed elevated triglycerides. The management involved maintaining anticoagulation with heparin dose-response testing and ensuring oxygenator function by measuring blood gas analysis, lactate, and arterial line pressure. The child recovered without complications. This case highlights the critical need for prompt identification and management of HTG in pediatric cardiac surgery to mitigate risks and ensure successful outcomes.

Influenza can lead to significant morbidity and mortality in children. Aortopathy tends to occur in adults during influenza season; however, aortic pathology in children with influenza is exceedingly rare. This report describes a child who experienced an aortic rupture during an influenza infection without evidence of secondary bacterial infection or connective tissue disorder.

Solitary myofibromas are rare, benign, mesenchymal tumors typically found in the head and neck regions. We report an exceptionally rare case of a solitary cardiac myofibroma and its short-term management and outcome. A two-month-old infant was referred due to a murmur. Echocardiography revealed a 2 × 1 cm solitary mass originating from a mitral papillary muscle, causing severe regurgitation. Magnetic resonance imaging showed the mass to be homogeneous on T1, hyperintense on T2, and hypointense on first-pass perfusion imaging with no enhancement on delayed postcontrast imaging. The mass was surgically excised partially due to its involvement with the mitral valve. Postoperative recovery was uneventful, with echocardiography four months later showing no tumor recurrence and trivial mitral regurgitation. This report provides valuable imaging and pathology examples to aid in the recognition of this rare tumor and demonstrates that successful surgical excision is possible, even with significant cardiac involvement.

We present a case of a 36 year old adult with anomalous origin of the right pulmonary artery from the aorta (hemitruncus), pulmonary hypertension, and moderate mitral regurgitation. Moreover, selective contrast echocardiogram (agitated saline injection into the right pulmonary artery) performed during preoperative cardiac catheterization demonstrated a moderate amount and prompt appearance of echo contrast in the left atrium (right pulmonary arteriovenous fistula). We successfully performed a reconstruction of the right pulmonary artery using a woven Dacron graft (12 mm in diameter). Six months postsurgery, the pulmonary arteriovenous fistula had substantially regressed, and the pulmonary arterial pressure and mitral regurgitation had both dramatically improved.

Nickel is a component of nitinol, an alloy used in several medical devices. Allergy to nickel may place patients at a high risk for severe hypersensitivity reactions. We report a rare case of a patient who developed severe ulcerative colitis ten years following closure of an atrial septal defect with the Amplatzer Septal Occluder device.