A 1-Day-Old Girl With Infantile Hemangioma and Sternal Cleft.

CASE PRESENTATION: A newborn girl presented to the hospital on the first day of life because of respiratory failure. She was born at home at 37 weeks' gestation with minimal prenatal care and was found to be small for gestational age. The patient was found to have partial sternal agenesis and sternal cleft, cutis aplasia, left facial hemangioma, micrognathia, wide-spaced nipples, and low-set ears. The mother's and baby's urine toxicology screening were positive for amphetamines. Chest radiographs on admission showed bilateral hazy opacities. CT scan of the chest showed an absent sternum with midline chest wall concavity. The patient was monitored preoperatively in the cardiac ICU for risks of arrythmia, respiratory failure, altered cardiac output, and acute cardiopulmonary decompensation.

Hypoplastic Left Heart Syndrome with Mitral Stenosis and Aortic Atresia-Echocardiographic Findings and Early Outcomes.

BACKGROUND: Mitral stenosis/aortic atresia (MS/AA) has been reported as a high-risk variant of hypoplastic left heart syndrome (HLHS), potentially related to ventriculocoronary connections (VCCs) or endocardial fibroelastosis (EFE) and myocardial hypoperfusion. We aimed to identify echocardiographic and clinical factors associated with early death or transplant in this group. METHODS: Patients with HLHS MS/AA treated at our center between 2000 and 2020 were included. Pre-stage I palliation echocardiograms were reviewed. Certain imaging factors, such as determination of VCC, EFE, and measurement of tricuspid annular plane systolic excursion were measured from retrospective review of preoperative images; others were derived from clinical reports. Groups were compared according to primary outcome of death or transplant prior to stage II palliation. RESULTS: Of 141 patients included, 39 (27.7%) experienced a primary outcome. Ventriculocoronary connections were identified in 103 (73.0%) patients and EFE in 95 (67.4%) patients. Among imaging variables, smaller ascending aorta size (median, 2.2 [interquartile range (IQR) 1.7-2.8] vs 2.6 [2.2-3.4] mm, P = .01) was associated with primary outcome. There was similar frequency of VCC (74.4% vs 72.5%, P = .83), EFE (59.0% vs 72.5%, P = .19), moderate or greater tricuspid regurgitation (5.1% vs 5.9%, P = 1.00), and similar right ventricular systolic function (indexed tricuspid annular plane systolic excursion 32.5 ± 7.3 vs 31.4 ± 7.2 mm/m2, P = .47) in the primary outcome group compared to other patients. Clinical factors associated with primary outcome included lower birth weight (mean, 2.8 ± SD 0.8 vs 3.3 ± 0.5 kg, P = .0003), gestational age <37 weeks (31.6% vs 4.9%, P < .0001), longer cardiopulmonary bypass time (median, 112 [IQR, 93-162] vs 82 [71-119] minutes, P = .001), longer intensive care unit length of stay (median, 19 [IQR, 10-30] vs 10 [7-15] days, P = .001), and extracorporeal membrane oxygenation following stage I palliation (43.6% vs 8.8%, P < .0001). Presence of VCCs and EFE was not associated with death or transplant after controlling for birth weight and era of stage I palliation. CONCLUSIONS: In one of the largest reported single-center cohorts of HLHS MS/AA, there were few pre-stage I palliation imaging characteristics associated with primary outcome. Imaging findings evaluated in this study, including the presence of VCC and/or EFE as determined using highly sensitive echocardiogram criteria, should not preclude intervention, although impact on long-term outcomes requires further evaluation.

Rheumatic mitral valve repair or replacement in the valve-in-valve era.

OBJECTIVE: For degenerative mitral disease, repair is superior to replacement; however, the best operative strategy for rheumatic mitral disease remains unclear. We evaluated the association between decision-making in choosing repair versus replacement and outcomes across 2 decades of rheumatic mitral surgery. METHODS: Patients undergoing isolated, first-time rheumatic mitral surgery were identified. Era 1 (1997-2008) and Era 2 (2009-2018) were distinguished by intraoperative assessment of anterior leaflet mobility/calcification (Era 2) in deciding between mitral repair versus replacement. Primary outcome was a composite of death, reoperation, and severe valve dysfunction. RESULTS: Among 180 patients, age was 59 ± 14 years, and ejection fraction was 58% ± 10%. A higher proportion in Era 1 (n = 56) compared with Era 2 (n = 124) had preoperative atrial fibrillation (68% vs 46%; P = .006); the groups were otherwise similar. Primary indication was mitral stenosis in 69% (124 out of 180; pure = 35, mixed = 89) and did not differ by era (P = .67). During Era 1, 70% (39 out of 56) underwent repair, compared with 33% (41 out of 124) during Era 2 (P < .001). Freedom from death, reoperation, or severe valve dysfunction at 5 years was higher in Era 2 (72% ± 9%) than Era 1 (54% ± 13%; P = .04). Five-year survival was higher in Era 2 than Era 1, but did not differ between repair versus replacement. Five-year cumulative incidence of reoperation with death as a competing risk did not differ by era, but was higher after repair than replacement. CONCLUSIONS: Careful assessment of anterior leaflet mobility/calcification to determine mitral repair or replacement was associated with improved outcomes. This decision-making strategy may alter the threshold for rheumatic mitral replacement in the current valve-in-valve era.

Inflammatory and Antioxidant Gene Transcripts: A Novel Profile in Postoperative Atrial Fibrillation.

Postoperative atrial fibrillation (POAF) is the most common complication after cardiac surgery; however, antiarrhythmic strategies have not lowered the rate of POAF. This study aimed to identify specific gene transcripts of atrial inflammation, inflammatory handling, and oxidative stress associated with POAF. Left atrial tissue was obtained from 50 patients undergoing intended degenerative mitral repair who did not have any of the following risk factors for POAF: history of atrial fibrillation or other arrhythmia, left atrial diameter greater than 6.0 cm, or left ventricular ejection fraction less than 40%. Postoperative outcomes and left atrial tissue messenger ribonucleuc acid (mRNA) levels were recorded. Parametric 2-sample t-tests and chi-square tests were used to evaluate for statistical significance in comparing POAF and non-POAF groups. Within 30 days of surgery, 19 of 50 of patients (38%) developed POAF. There were no significant preoperative, intraoperative, or postoperative differences between POAF and non-POAF patients. In the tissue transcriptome analysis, POAF patients were found to have a worse preoperative inflammatory state with higher levels of tumor necrosis factor alpha, Interleukin-6, and nuclear factor of kappa light polypeptide gene enhancer in B-cells mRNA, worse inflammatory handling capacity with lower levels of nuclear factor of kappa light polypeptide gene enhancer in B-cells inhibitor mRNA, and reduced antioxidant defenses with lower levels of glutathione synthetase, glutathione reductase, and mitochondrial superoxide dismutase 2 mRNA. This study found POAF patients to have preoperative left atrial tissue profiles suggestive of more inflammation, worse inflammatory handling, and reduced antioxidant defenses against oxidative stress. Investigation of therapies targeted to the tissue-specific inflammatory transcriptome of POAF patients is warranted.

Short-term outcomes of a simple and effective approach to aortic root and arch repair in acute type A aortic dissection.

OBJECTIVE: To evaluate short-term outcomes following direct aortic root and arch repair in patients with acute type A aortic dissection (ATAAD) without technical adjuncts. METHODS: Between 2012 and 2016, 94 consecutive patients with ATAAD underwent surgical repair, including aortic root repair (n = 45), root replacement (n = 39), or no root procedure (n = 10). Aortic root repair was achieved by running approximation of the dissected aortic wall circumferentially at the sinotubular junction and reinforcing the coronary ostia with 5-0 Prolene. The aortic root and arch were anastomosed to the Dacron graft with 5-0 Prolene without Teflon felt or biological glue. RESULTS: Postoperative new-onset myocardial infarction, stroke, renal failure, and complete heart block occurred in 0%, 4%, 13%, and 0% of patients, respectively, whereas 30-day mortality was 4%. The incidences of permanent neurologic deficit and renal failure were 1% and 2%, respectively. Up to 5 years, the aortic root repair group was free from residual or recurrent aortic root dissection, major change in the aortic root diameter, and moderate to severe aortic regurgitation; the entire cohort was free of anastomotic pseudoaneurysm and reoperation for proximal aortic pathology or significant change in diameter of the aortic arch and descending thoracic aorta. Overall survival was 85% at 4 years and was significantly enhanced in the aortic root repair group compared with the Bentall group (n = 24) (93% vs 57%; P = .035). CONCLUSIONS: Direct aortic root and arch repair with approximation of the aortic wall without use of technical adjuncts is safe and effective for patients with ATAAD. If warranted, preservation of the native aortic valve should be considered for a potential survival benefit.

Effect of sex and race on outcome in patients undergoing congenital heart surgery: an analysis of the society of thoracic surgeons congenital heart surgery database.

BACKGROUND: Previous studies on the impact of race and sex on outcome in children undergoing cardiac operations were based on analyses of administrative claims data. This study uses clinical registry data to examine potential associations of sex and race with outcomes in congenital cardiac operations, including in-hospital mortality, postoperative length of stay (LOS), and complications. METHODS: The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) was queried for patients younger than 18 years undergoing cardiac operations from 2007 to 2009. Preoperative, operative, and outcome data were collected on 20,399 patients from 49 centers. In multivariable analysis, the association of race and sex with outcome was examined, adjusting for patient characteristics, operative risk (Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery [STAT] mortality category), and operating center. RESULTS: Median age at operation was 0.4 years (interquartile range 0.1-3.4 years), and 54.4% of patients were boys. Race/ethnicity included 54.9% white, 17.1% black, 16.4% Hispanic, and 11.7% "other." In adjusted analysis, black patients had significantly higher in-hospital mortality (odds ratio [OR], 1.67; 95% confidence interval [CI], 1.37-2.04; p<0.001) and complication rate (OR, 1.15; 95% CI, 1.04-1.26; p<0.01) in comparison with white patients. There was no significant difference in mortality or complications by sex. Girls had a shorter LOS than boys (-0.8 days; p<0.001), whereas black (+2.4 days; p<0.001) and Hispanic patients (0.9 days; p<0.01) had longer a LOS compared with white patients. CONCLUSIONS: These data suggest that black children have higher mortality, a longer LOS, and an increased complication rate. Girls had outcomes similar to those of boys but with a shorter LOS of almost a day. Further study of potential causes underlying these race and sex differences is warranted.

A method of transcutaneously adjustable pulmonary artery banding for staged left ventricular retraining.

OBJECTIVES: The placement of a pulmonary artery band to retrain the left ventricle often requires reoperation for band adjustment. We describe an effective technique for the placement of a transcutaneously adjustable pulmonary artery band that allows adjustments to be made without the need for repeat sternotomy. METHODS: Using standard catheters, an adjustable band was fashioned and placed around the pulmonary artery with the control end positioned in the subcutaneous tissue of the anterior chest wall. Tightening or loosening of the band can be subsequently performed by exposing the control end without the need for reopening the chest. RESULTS: From 1995 to 2011, 11 patients underwent placement of a transcutaneously adjustable pulmonary artery band for the purpose of retraining the morphologic left ventricle for a subsequent arterial switch operation. One or more band adjustments were required in 6 patients (1 loosened and 5 tightened, 55%) at a mean of 281 days (median, 98; range, 0-917) after initial band placement. All were accomplished successfully by exposing the band in the subcutaneous tissue of the anterior chest wall and performing the adjustment under Doppler echocardiographic guidance. CONCLUSIONS: This technique affords the operating surgeon the freedom to apply the band very gradually, erring on the side of safety, and facilitates a gradual increase in ventricular afterload that can be performed as a minor procedure.