Critical COVID-19 Complicating Recovery From Surgical Repair of Congenital Heart Disease.

This case highlights the need for accurate and rapid testing for severe acute respiratory syndrome coronavirus 2 and also underscores the need for caregivers to remain vigilant for coronavirus disease 2019 in the postoperative setting despite negative preoperative testing.

Thoracoscopic Resection of Left Atrial Appendage Aneurysm in a 16-Year-Old Boy.

We report a case of a left atrial appendage aneurysm (LAAA) in a 16-year-old boy presenting with supraventricular tachycardia (SVT). The aneurysm was detected incidentally on a routine echocardiogram performed before an electrophysiology study for evaluation and management of the SVT. The aneurysm was successfully resected under cardiopulmonary bypass through video-assisted thoracoscopic surgery. This type of surgery is a useful approach for LAAA in the pediatric population.

Successful 2-Stage Pacemaker Implantation for Atrioventricular Block in a Low-Birth-Weight Infant with Congenital Heart Disease: Case Report.

Congenital heart block is a potentially life-threatening condition with high morbidity and mortality, especially in the presence of congenital heart disease. We present the case of a low-body-weight premature infant with complex single ventricle congenital heart disease and high-grade atrioventricular block. A 2-staged pacing approach provided atrio-ventricular synchrony and allowed her to grow until a permanent dual-chamber pacemaker system could be implanted.

Thoracic duct embolization in kaposiform lymphangiomatosis.

An 11-year-old girl with kaposiform lymphangiomatosis presented with recurrent chylous pericardial effusions that were refractory to pericardial drainage and medical therapy. Magnetic resonance imaging demonstrated a prominent lymphatic duct with anterior mediastinal extension into the left clavicular region and a region of high signal that was favored to represent a low-flow lymphatic malformation. The patient underwent direct access thoracic duct lymphangiography with thoracic duct embolization and sclerotherapy of the large left-sided neck and pericardial lymphatic malformation. After the procedure, her pericardial effusions resolved, and she has remained asymptomatic for 15 months.

Reanastomosis and rehabilitation of interrupted left pulmonary artery derived from atretic ductus.

True absence of a branch pulmonary artery is rare. We identified a patient initially diagnosed with an absent left pulmonary artery at a previous hospital. Due to disagreement in the initial diagnosis, she had a diagnostic catheterisation, which revealed an isolated left pulmonary artery off the left innominate artery via a ductus. The ductus was recanalised with serial stenting and balloon dilatation followed by reanastomosis to the main pulmonary artery. In a patient who initially is diagnosed with an absent pulmonary artery, an alternative diagnosis, such as this case report, should be considered.

An intra-aortic guide wire: what "knot" to do.

We report a rare complication of central venous catheter placement in a 5-month-old baby.

Medium-Term Outcomes After Implantation of Expanded Polytetrafluoroethylene Valved Conduit.

BACKGROUND: Pulmonary valve replacement is becoming the most frequent congenital heart surgery performed on adolescents and young adults. Numerous surgical options are available, including autologous pericardium, mechanical valves, allografts, and bioprosthetic valves. Each option has limitations with durability and freedom from reintervention for stenosis or insufficiency, particularly in the pediatric population. The purpose of this study was to analyze our uniquely designed, expanded polytetrafluoroethylene (ePTFE) valved conduit (VC) for right ventricular outflow tract reconstruction. METHODS: Beginning in 2012, ePTFE VC were implanted in 26 patients with a median age of 9.8 years (range, 1.0 to 15.9). Bicuspid VC were used in the first 3 patients, and tricuspid VC were used in 23 patients. The ePTFE VC is fashioned from commercially available ePTFE tube graft (16 mm in 1 patient, 20 mm in 7 patients, 24 mm in 18 patients) and 0.1 mm thick ePTFE membrane for the leaflet material. Valve function was assessed by echocardiogram after the implantation. RESULTS: There were no postoperative hospital deaths. Mean follow-up was 2.6 years (range, 4 months to 4.7 years), and there have been no reoperations for the ePTFE VC. There was one reintervention for stenosis at the distal anastomosis. Pulmonary insufficiency was mild or less in 24 patients (92%). The average peak instantaneous pressure gradient between the right ventricle and the pulmonary artery was 22.4 ± 15.1 mm Hg at latest follow-up. CONCLUSIONS: Compared with historical data for other pulmonary valve replacement options, our ePTFE VC shows superior medium-term performance, with less reintervention or significant valve dysfunction.

Timing of adding blood to prime affects inflammatory response to neonatal cardiopulmonary bypass.

Complications from systemic inflammation are reported in neonates following exposure to cardiopulmonary bypass. Although the use of asanguinous primes can reduce these complications, in neonates, this can result in significant haemodilution, requiring addition of blood. This study investigates whether the addition of blood after institution of bypass alters the inflammatory response compared with a blood prime. Neonatal swine were randomised into four groups: blood prime, blood after bypass but before cooling, blood after cooling but before low flow, and blood after re-warming. All groups were placed on central bypass, cooled, underwent low flow, and then re-warmed for a total bypass time of 2 hours. Although haematocrit values between groups varied throughout bypass, all groups ended with a similar value. Although they spent time with a lower haematocrit, asanguinous prime groups did not have elevated lactate levels at the end of bypass compared with blood prime. Asanguinous primes released less tumour necrosis factor α than blood primes (p=0.023). Asanguinous primes with blood added on bypass produced less interleukin 10 and tumour necrosis factor α (p=0.006, 0.019). Animals receiving blood while cool also showed less interleukin 10 and tumour necrosis factor α production than those that received blood warm (p=0.026, 0.033). Asanguinous primes exhibited less oedema than blood primes, with the least body weight gain noted in the end cool group (p=0.011). This study suggests that using an asanguinous prime for neonates being cooled to deep hypothermia is practical, and the later addition of blood reduces inflammation.

Development of chylous ascites in an infant with ventricular septal defect: a case report.

This case report describes chylous ascites associated with a CHD in a 4-month-old infant. Although atraumatic chylous ascites are a rare clinical finding, the recognition and treatment of chylous ascites influence the timing of cardiac surgery.

Ross procedure for patient with Marfan syndrome.

The most prominent long-term complication after the Ross procedure is the risk of autograft dilatation, and therefore its application in patients at increased perceived risk of autograft dilatation (those with bicuspid aortic valve disease, aortic insufficiency [AI] with dilated aorta, collagen vascular diseases such as Marfan syndrome) has been discouraged. We reported a modified Ross procedure in 2005 in which the autograft was completely encased in a polyester graft before implantation to prevent further dilatation of the autograft. This case report describes follow-up of a patient with Marfan syndrome who underwent this modified Ross procedure in July 2005.

A modification of the pulmonary autograft procedure to prevent late autograft dilatation.

Although the pulmonary autograft procedure for aortic valve replacement is a commonly utilized option for children, its use is diminishing in adult-aged patients. One commonly cited concern is the tendency for the pulmonary autograft to dilate in the aortic position. This article reviews a technique we have used in 36 patients since October, 2004 that stabilizes the autograft so that it cannot dilate. There have been no operative or late deaths and the autograft has continued to function in 34 patients. Two patients have undergone autograft replacement because of early failure, which we believe was likely related to technical considerations in our early technique (first reported in the 2005 STCVS Pediatric Cardiac Surgery Annual). The technical modifications described in this article have produced a more reliable and reproducible technique and have not resulted in any autograft failures in our experience. One patient with Marfan's syndrome and a bicuspid aortic valve is symptom- and dilation-free 8 years post op, with no autograft or pulmonary homograft insufficiency, normal activity and a stable aortic root by serial echocardiography. Our results suggest that this technique might be applicable for selected adult patients in whom autograft growth is not necessary and for whom the risk of autograft dilatation would provide a reason to avoid a pulmonary autograft procedure.

Periarteritis in lung from a continuous-flow right ventricular assist device: role of the local Renin-Angiotensin system.

BACKGROUND: We previously reported renal arterial periarteritis after implantation of a continuous-flow left ventricular assist device in calves. The purpose of the present study was to investigate whether the same periarteritis changes occur in the intrapulmonary arteries after implantation of a continuous-flow right ventricular assist device (CFRVAD) in calves and to determine the mechanism of those histologic changes. METHODS: Ten calves were implanted with a CFRVAD for 29 ± 7 days, and we compared pulmonary artery samples and hemodynamic data before and after CFRVAD implantation prospectively. RESULTS: After implantation, the pulsatility index (pulmonary arterial pulse pressure/pulmonary arterial mean pressure) significantly decreased (0.88 ± 0.40 before vs 0.51 ± 0.22 after; p < 0.05), with severe periarteritis of the intrapulmonary arteries in all animals. Periarterial pathology included hyperplasia and inflammatory cell infiltration. The number of inflammatory cells positive for the angiotensin II type 1 receptor was significantly higher after implantation (7.8 ± 6.5 pre-CFRVAD vs 313.2 ± 145.2 at autopsy; p < 0.01). Serum angiotensin-converting enzyme activity significantly decreased after implantation from 100% to 49.7 ± 17.7% at week 1 (p = 0.01). Tissue levels of angiotensin-converting enzyme also demonstrated a significant reduction (0.381 ± 0.232 before implantation vs 0.123 ± 0.096 at autopsy; p = 0.043). CONCLUSIONS: Periarteritis occurred in the intrapulmonary arteries of calves after CFRVAD implantation. The local renin-angiotensin system (not the angiotensin-converting enzyme pathway) plays an important role in such changes.

Contemporary patterns of surgery and outcomes for aortic coarctation: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.

OBJECTIVE: The objective of this study was to describe characteristics and early outcomes across a large multicenter cohort undergoing coarctation or hypoplastic aortic arch repair. METHODS: Patients undergoing coarctation or hypoplastic aortic arch repair (2006-2010) as their first cardiovascular operation in the Society of Thoracic Surgeons Congenital Heart Surgery Database were included. Group 1 patients consisted of those with coarctation or hypoplastic aortic arch without ventricular septal defect (coarctation or hypoplastic aortic arch, isolated); group 2, coarctation or hypoplastic aortic arch with ventricular septal defect (coarctation or hypoplastic aortic arch, ventricular septal defect); and group 3, coarctation or hypoplastic aortic arch with other major cardiac diagnoses (coarctation or hypoplastic aortic arch, other). RESULTS: The cohort included 5025 patients (95 centers): group 1, 2705 (54%); group 2, 840 (17%); and group 3, 1480 (29%). Group 1 underwent coarctation or hypoplastic aortic arch repair at an older age than groups 2 and 3 (groups 1, 2, and 3, 75%, 99%, and 88% <1 year old, respectively; P < .0001). The most common operative techniques for coarctation or hypoplastic aortic arch repair (group 1) were end-to-end (33%) or extended end-to-end (56%) anastomosis. Overall mortality was 2.4%, and was 1%, 2.5%, and 4.8% for groups 1, 2, and 3 respectively (P < .0001). Ventricular septal defect management strategies for group 2 patients included ventricular septal defect closure (n = 211, 25%), pulmonary artery band (n = 89, 11%), or no intervention (n = 540, 64%) without significant difference in mortality (4%, 1%, 2%; P = .15). Postoperative complications occurred in 36% of patients overall and were more common in groups 2 and 3. There were no occurrences of spinal cord injury (0/973). CONCLUSIONS: In the current era, primary coarctation or hypoplastic aortic arch repair is performed predominantly in neonates and infants. Overall mortality is low, although those with concomitant defects are at risk for higher morbidity and mortality. The risk of spinal cord injury is lower than previously reported.

Valve-sparing replacement of the aortic root after repair of tetralogy of Fallot.

Progressive aortic root dilatation is a common feature after surgical repair of tetralogy of Fallot. This report describes a successful valve-sparing replacement of the aortic root in a patient with significant dilated aortic root and aortic regurgitation after repair of tetralogy of Fallot.

Neonatal ascending aortic thrombus: successful medical treatment.

Neonatal aortic thrombosis is a potentially life-threatening condition with significant morbidity and mortality if undiagnosed and untreated. The most common location of arterial thrombosis in neonates is in the abdominal aorta and is associated with umbilical artery catheterisation. There are only a few previous reports of thrombosis in the ascending aorta. We describe a case of ascending aortic thrombosis in a neonate who underwent successful thrombolytic therapy.

Pulmonary artery sling and tracheal bronchus presenting in a 2-year-old child.

Pulmonary artery sling is an incomplete vascular ring, the result of the left pulmonary artery arising from the right pulmonary artery and effectively constricting the airway, and it usually presents within the first weeks to months of life. We report a surgical correction of tracheal stenosis for a two-year-old patient associated with pulmonary artery sling and tracheal broncus.

Anomalous left coronary artery from the pulmonary arteryin an adult.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect that usually presents before the age of 1 year. Several surgical options exist for the correction of ALCAPA; however, debate continues regarding the optimal repair technique in adult populations. We report the case of successful surgical repair of ALCAPA with a direct aortic implantation technique in a 44-year-old mother of 4 children.

Pentalogy of Cantrell with double-outlet right ventricle: a case of surgical correction.

Pentalogy of Cantrell is characterised by a combination of severe defects in the middle of the chest including the sternum, diaphragm, heart, and abdominal wall. Mortality rate after cardiac surgery is usually high. We report a successful total correction of the cardiac defects in a case of Pentalogy of Cantrell with a double-outlet right ventricle prior to abdominal wall defect repair.