Is Cardiac Surgery Safe during Pregnancy? A 40-Year Single Institution Experience.

BACKGROUND: Limited data exist to characterize maternal and fetal outcomes during pregnancy using cardiopulmonary bypass. METHODS: Retrospective review was performed of all pregnant individuals who underwent cardiac surgery utilizing cardiopulmonary bypass at a single center from 1978 to 2023. Descriptive statistical analysis was performed, with a median reported for continuous variables and incidence for dichotomous variables. RESULTS: Twenty-nine pregnant patients with a median age of 28 (25, 32) years underwent cardiac surgery using cardiopulmonary bypass at a median gestation of 25 (16, 29) weeks. Surgery was performed in the 1st trimester for 3 (10%), 2nd trimester for 16 (55%), and 3rd trimester for 10 (35%) patients; 15 (52%) were emergent and 14 (48%) were urgent procedures. There was one (3%) maternal death two days after mechanical aortic valve thrombectomy and 5 (17%) fetal losses. Fourteen patients who underwent cardiac surgery using cardiopulmonary bypass with continuing pregnancy experienced a 29% fetal mortality rate, and 7 patients underwent delivery prior to surgery and experienced 14% fetal mortality. Among cases of fetal loss, surgery was performed at a median of 25 (21, 26) weeks compared to a median of 23 (20, 29) weeks in cases without fetal loss (p=0.55). CONCLUSIONS: Cardiac surgery during pregnancy was associated with low maternal mortality but significant fetal mortality. This single-institution series supports consideration of cesarean delivery prior to cardiopulmonary bypass procedures if the fetus is of a viable gestational age to minimize mortality.

Adult Congenital Heart Disease Transplantation: Does Univentricle Physiology Impact Early Mortality?

BACKGROUND: With patients with congenital heart disease increasingly living into adulthood, there is a growing population of patients with adult congenital heart disease (ACHD) who have heart failure. Limited data exist on evaluating heart transplantation in this population. METHODS: A retrospective review was performed of patients with ACHD who underwent heart transplantation from November 1990 to January 2023. Kaplan-Meier, cumulative incidence accounting for competing risk of death, and subgroup analyses comparing those patients with biventricular (BiV) and univentricular (UniV) physiology were performed. Data are presented as median (interquartile range [IQR]) or counts (%). RESULTS: A total of 77 patients with a median age of 36 years (IQR, 27-45 years) were identified, including 57 (74%) BiV and 20 (26%) UniV patients. Preoperatively, UniV patients were more likely to have cirrhosis (9 of 20 [45.0%] vs 4 of 57 [7.0%]; P < .001) and protein losing enteropathy (4 of 20 [20.0%] vs 1 of 57 [1.8%]; P = .015). Multiorgan transplantation was performed in 23 patients (30%) and more frequently in UniV patients (10 [50%] vs 13 [23%]; P = .04). Operative mortality was 6.5%, 2 of 20 (10%) among UniV patients and 2 of 57 (4%) among BiV patients (P = .276). Median clinical follow-up was 6.0 years (IQR, 1.4-13.1 years). Survival tended to be lower among UniV patients compared with BiV patients, particularly within the first year (P = .09), but it was similar for survivors beyond 1 year. At 5 years, the incidence of rejection was 28% (IQR, 17%-38%) and that of coronary allograft vasculopathy was 16% (IQR, 7%-24%). CONCLUSIONS: Underlying liver disease and the need for heart-liver transplantation were significantly higher among UniV patients. Survival tended to be lower among UniV patients, particularly within the first year, but it was similar for survivors beyond 1 year.

Pediatric Quadricuspid Aortic Valve: Morphology, Characteristics, Clinical Outcomes, and Literature Review.

BACKGROUND: Quadricuspid aortic valve (QAV) is an exceedingly rare congenital heart defect (CHD) which has not been well-defined in a pediatric population. METHODS: The Mayo Clinic echocardiography database was retrospectively analyzed to identify patients ≤18 years diagnosed with QAV from January 1990 to December 2023. Patients with truncus arteriosus were excluded. All images were independently reviewed to define morphology of the QAV by using the Hurwitz and Roberts classification. RESULTS: Fourteen patients with QAV were identified with a median age at time of diagnosis being 10.5 years (interquartile range [IQR] 6-14 years). Male-to-female ratio was 3:1. Associated CHDs were present in 50% (n = 7) patients. The most common morphological subtypes of QAV were Type D in 43% (n = 6) and Type B in 29% (n = 4). Aortic regurgitation was the most frequently associated valvular abnormality affecting 86% (n = 12) cases, with greater than moderate regurgitation in only two patients. Aortic valve stenosis was observed in 14% (n = 2) patients. Ascending aortic dilatation was present in 21% (3/14) of the cohort, but only 14% (1/7) of isolated QAV patients. At a mean follow up of 11 ± 6.6 years and a median follow-up age of 22 years (IQR 14-27 years), survival was 100% with no primary interventions on the aortic valve or aorta. However, four patients required surgical interventions for associated CHDs. CONCLUSION: Among children with QAV, almost half of the patients had additional CHD. Aortic regurgitation was the predominant hemodynamic abnormality. Long-term survival was excellent with minimal progression during childhood and adolescence.

PA-VSD Without MAPCA(s): Review of Long-Term Outcomes and Reinterventions.

BACKGROUND: Pulmonary atresia with ventricular septal defect without major aortopulmonary collateral arteries (MAPCAs) is an uncommon form of congenital heart disease. As more patients with congenital heart disease live to adulthood, the objective of this article was to review the long-term results of this specific population. METHODS: A review of the PubMed database was performed using pertinent key words (pulmonary atresia, tetralogy of Fallot, conduit, right ventricle-pulmonary artery) concentrating on studies from 1990-present and published in English. Most studies of pulmonary atresia-ventricular septal defect patients included those with and without MAPCAs. Analysis included examination of the entire cohort, consideration of the proportion of patients with MAPCAs, and any subgroup analysis of the patients without MAPCAs. RESULTS: Survival is approximately 80% at ten years and is improved with complete repair and larger pulmonary arteries. Some studies have found genetic syndromes and extracardiac anomalies to impact survival, while others have not. Incomplete repair has been shown to be associated with worse survival. Independent of initial management strategy, patients with pulmonary atresia and ventricular septal defects without MAPCAs require repeat intervention on the right ventricular outflow tract. Hypoplastic pulmonary arteries have been shown to be a risk factor for reintervention, and decreased conduit durability has been shown with younger age at implantation of conduit. CONCLUSIONS: Long-term outcomes have improved for patients with pulmonary atresia-ventricular septal defect without MAPCAs, with complete repair and adequate pulmonary arteries favorable for survival. Long-term outcomes include reinterventions, both catheter-based and surgical, predominantly on the right ventricular outflow tract.

Outcomes following multivalve reoperation in adults with congenital heart disease: A 30-year, single-center study.

OBJECTIVE: As patients with congenital heart disease increasingly live into adulthood, reoperative surgery is frequently required. Although half of these are valve-related procedures, little is known regarding early and late outcomes, and factors associated with adverse outcomes. METHODS: From 1993 to 2022, a total of 1960 adult patients with congenital heart disease underwent repeat median sternotomy at our institution. Of these, 502 patients (26%) underwent intervention on 2 or more valves and constituted the study cohort. RESULTS: The median age was 39 (27-51) years, and 275 patients (55%) were female. A second sternotomy was performed in 265 patients (53%), a third sternotomy was performed in 135 patients (27%), a fourth sternotomy was performed in 75 patients (15%), and a fifth or more sternotomy was performed in 27 patients (5%). Interventions were performed on 2 valves in 436 patients (87%), 3 valves in 62 patients (12%), and 4 valves in 4 patients (1%). The most common combinations were pulmonary and tricuspid in 241 patients (48%), followed by mitral and tricuspid in 85 patients (17%), aortic and pulmonary in 42 patients (8%), and aortic and mitral in 41 patients (8%). Early mortality was 4.2% overall and 2.7% for elective operations. Nonelective operations and congenital heart disease of major complexity were independently associated with early mortality. Median follow-up was 14 years. One, 5-, and 10-year survivals were 93.6%, 89.3%, and 79.5%, respectively. Factors independently associated with overall mortality were age, ventricular dysfunction, coronary artery disease, renal failure, double valve replacement, nonelective operations, and bypass time. CONCLUSIONS: Multiple valve interventions are common and confer low early mortality in the elective setting. Referral before ventricular dysfunction and in an elective setting optimizes outcomes.

Risk of Cardiac Reoperation: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

BACKGROUND: The increasing number of congenital heart disease patients undergoing reoperative cardiac surgery presents critical and growing challenges. Our objective was to evaluate the association between the number of prior cardiopulmonary bypass operations and operative mortality and morbidity in a national cohort. METHODS: The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) was reviewed for index cardiac operations on cardiopulmonary bypass during 2016 to 2021. Infants and patients with functionally univentricular physiology were excluded. Multivariable logistic regression adjusted for covariates in the STS-CHSD Mortality Risk Model, the STS-European Association for Cardio-Thoracic Surgery (STAT) Mortality Category, and institutional volume. RESULTS: Of 50,625 eligible operations, 22,100 (44%) were performed on patients with ≥1 prior cardiopulmonary bypass operations. Most common diagnoses were tetralogy of Fallot (4340 of 22,100 [19.6%]), pulmonary atresia/ventricular septal defect (1334 of 22,100 [6.0%]), and aortic stenosis (966 of 22,100 [4.4%]). Operative mortality correlated with number of prior cardiopulmonary bypass operations: 157 of 28,525 (0.6%) for 0, 127 of 13,488 (0.9%) for 1, 81 of 5,664 (1.4%) for 2, 61 of 2039 (3.0%) for 3, 35 of 623 (5.6%) for 4, 10 of 207 (4.8%) for 5, and 5 of 79 (6.3%) for ≥6 operations (P < .001). On multivariable analysis, patients with ≥3 prior cardiopulmonary bypass operations had higher risk of operative mortality (odds ratio, 2.31; P < .001) and major morbidity (odds ratio, 1.60; P < .001). Annual institutional volume and age were not associated with either outcome. CONCLUSIONS: Three or more prior cardiopulmonary bypass operations was an independent risk factor for operative mortality/morbidity, even after controlling for risk factors and institutional volume. Future research is needed to identify modifiable factors to optimize outcomes, particularly for those with ≥3 prior cardiopulmonary bypass operations.

The Society of Thoracic Surgeons Clinical Practice Guidelines on the Management of Neonates and Infants With Coarctation.

BACKGROUND: Although coarctation of the aorta without concomitant intracardiac pathology is relatively common, there is lack of guidance regarding aspects of its management in neonates and infants. METHODS: A panel of experienced congenital cardiac surgeons, cardiologists, and intensivists was created, and key questions related to the management of isolated coarctation in neonates and infants were formed using the PICO (Patients/Population, Intervention, Comparison/Control, Outcome) Framework. A literature search was then performed for each question. Practice guidelines were developed with classification of recommendation and level of evidence using a modified Delphi method. RESULTS: For neonates and infants with isolated coarctation, surgery is indicated in the absence of obvious surgical contraindications. For patients with risk factors for surgery, medical management before intervention is reasonable. For those stable off prostaglandin E1, the threshold for intervention remains unclear. Thoracotomy is indicated when arch hypoplasia is not present. Sternotomy is preferable when arch hypoplasia is present that cannot be adequately addressed through a thoracotomy. Sternotomy may also be considered in the presence of a bovine aortic arch. Antegrade cerebral perfusion may be reasonable when the repair is performed through a sternotomy. Extended end-to-end, arch advancement, and patch augmentation are all reasonable techniques. CONCLUSIONS: Surgery remains the standard of care for the management of isolated coarctation in neonates and infants. Depending on degree and location, arch hypoplasia may require a sternotomy approach as opposed to a thoracotomy approach. Significant opportunities remain to better delineate management in these patients.

Delayed Sternal Closure vs Emergency Sternal Reopening in Adults With Congenital Heart Disease.

BACKGROUND: Limited data exist regarding outcomes of delayed sternal closure (DSC) in adults with congenital heart disease (ACHD). METHODS: We reviewed 159 ACHD patients undergoing cardiac operation from 1993 to 2023 who required DSC (open sternum at the end of operation, n = 112) or sternum emergently reopened (n = 47). Regression models were performed to determine factors associated with outcomes. RESULTS: Of 112 patients undergoing DSC, 87 patients (77.6%) underwent DSC ≤4 days and 25 patients (22.3%) >4 days. The most common operations were valve (n = 35 [31.2%]), aortic (n = 33 [29.4%]), and right ventricular outflow tract procedures (n = 23 [20.5%]). Median time to chest closure was 2 days (interquartile range, 1-5 days). Apart from sex, baseline characteristics were similar between DSC groups. A stepwise increase in early mortality was observed from DSC ≤4 days to DSC >4 days (6.8% vs 32%), as well as the incidence of early complications, except sternal infection. Risk factors associated with early mortality were age (P = .02), DSC >4 days (P < .001), hemodynamic indication (P = .03), and single ventricle (P = .02). On multivariable analysis, lower ejection fraction (P = .04), hemodynamic indication (P = .02), single ventricle (P = .004), and diabetes mellitus (P = .03) were predictors of prolonged time to chest closure. Among hospital survivors, late survival was similar between patients undergoing DSC ≤4 days vs >4 days (P = .48). CONCLUSIONS: A brief duration of DSC in ACHD patients is associated with low morbidity and mortality. Higher early mortality and complications were observed among patients who did not achieve chest closure within 4 days.

Heart Transplantation for Ebstein Anomaly due to Late Surgical Presentation.

Ebstein anomaly is a myopathy of the right ventricle characterized by failure of tricuspid valve delamination with accompanying tricuspid regurgitation. Its rarity, considerable anatomic variability, and frequent absence of symptoms can make the timing of surgery challenging. Contemporary tricuspid repair techniques can be performed with low mortality and bidirectional cavopulmonary anastomosis reduces the risk of operation when right ventricular function is poor. Here, we present a patient who presented late for surgery, failed high-risk conventional surgery and required heart transplantation.

Hypertrophic Cardiomyopathy: Preadolescence, Mitral Valve Disease, and Midventricular Obstruction.

Septal myectomy is indicated in patients with obstructive hypertrophic cardiomyopathy (HCM) who have persistent symptoms despite medical therapy, intolerance of medication side effects, or severe resting or provocable gradients. Septal myectomy at high volume centers is safe, with low operative mortality (1%) and low rates of complications such as complete heart block or ventricular septal defect (3% and 0.5%, respectively). Additionally, improved survival following myectomy has been observed when compared to patients with obstructive HCM managed medically or those with nonobstructive HCM. As a longstanding, quaternary referral center for septal myectomy, our institution has built significant experience and expertise in the surgical and medical management of HCM, including atypical HCM, defined as preadolescent patients, those with mitral valve disease, and those with isolated midventricular obstruction. The most important factor of septal myectomy in achieving complete resolution of obstruction and avoiding recurrence is the apical extent of the myectomy trough, which must extend to the septum opposite the papillary muscles. If this cannot be fully achieved via a transaortic exposure, especially in preadolescents and patients with midventricular obstruction, then a transapical approach may be needed. Mitral valve repair is rarely necessary as SAM-mediated MR resolves with adequate myectomy alone, but mitral repair is performed in cases of intrinsic valvular disease. In this manuscript we provide a summary of current operative techniques and outcomes data from our institution on the management of these various categories of HCM.

Anomalous Aortic Origin of a Coronary Artery.

BACKGROUND: Although anomalous aortic origin of a coronary artery (AAOCA) is associated with risk of sudden cardiac arrest (SCA), there is a spectrum of disease, with the appropriate management for many remaining unclear. Increasing data warrant review for an updated perspective on management. METHODS: A panel of congenital cardiac surgeons, cardiologists, and imaging practitioners reviewed the current literature related to AAOCA and its management. Survey of relevant publications from 2010 to the present in PubMed was performed. RESULTS: The prevalence of AAOCA is 0.4% to 0.8%. Anomalous left coronary artery is 3 to 8 times less common than anomalous right coronary, but carries a much higher risk of SCA. Nevertheless, anomalous right coronary is not completely benign; 10% demonstrate ischemia, and it remains an important cause of SCA. Decision-making regarding which patients should be recommended for surgical intervention includes determining anatomic features associated with ischemia, evidence of ischemia on provocative testing, and concerning cardiovascular symptoms. Ischemia testing continues to prove challenging with low sensitivity and specificity, but the utility of new modalities is an active area of research. Surgical interventions focus on creating an unobstructed path for blood flow and choosing the appropriate surgical technique given the anatomy to accomplish this. Nontrivial morbidity has been reported with surgery, including new-onset ischemia. CONCLUSIONS: A proportion of patients with AAOCA demonstrate features and ischemia that warrant surgical intervention. Continued work remains to improve the ability to detect inducible ischemia, to risk stratify these patients, and to provide guidance in terms of which patients warrant surgical intervention.

Valve-Sparing Aortic Root Replacement State-of-the-Art Review, Part II: Surgical Techniques.

Aortic valve disease is common, and valve-preserving operations are preferred whenever possible. Valve-sparing aortic root replacement (VSRR) has become an important tool for managing aortic root pathology in children and adults. The learning curve for this operation is challenging, but with increasing experience and technical modifications, early and late outcomes continue to improve. Durable long term results vary based on underlying anatomy, pathology, and patient selection, as well as surgeon expertise. Part II of this VSRR State-of-the-Art Review article provides technical pearls related to VSRR.

Valve-Sparing Aortic Root Replacement State-of-the-Art Review, Part I: Anatomy and Physiology.

Aortic valve disease is common, and valve-preserving operations are preferred whenever possible. Valve-sparing aortic root replacement has become an important tool for managing aortic root pathology in children and adults. The learning curve for this operation is challenging, but with increasing experience and technical modifications, early and late outcomes continue to improve. Durable long-term results vary based on the underlying anatomy, pathology, and patient selection, as well as surgeon expertise. The first installment of this Valve-Sparing Aortic Root Replacement State-of-the-Art Review article addresses patient anatomy and physiology as it relates to candidacy for VSRR.

National In-Hospital Outcomes of Mechanical Mitral Valve Replacement in the Pediatric Population.

Background: National data about the outcomes of children undergoing mechanical mitral valve replacement (m-MVR) are scarce. Methods: A retrospective review of hospitalizations from the Kids' Inpatient Database was performed for patients ≤18 years of age in the United States. A total of 500 patients underwent m-MVR in 2009, 2012, 2016, and 2019. Patients with single ventricle physiology were excluded (n = 13). These patients were categorized into three groups according to age: neonates (<1 month, n = 20), infants (1-12 months, n = 76 patients), and children (1-18 years, n = 404). Outcomes were compared between the three groups. Results: The proportion of m-MVR involving children undergoing MV procedures (repair and replacement) has increased from 17.3% in 2009 to 30.8% in 2019 (Ptrend < .01). History of cardiac surgery was present in 256 patients (51.2%). Concomitant procedures were performed in 119 patients (23.8%). Intra- or postoperative extracorporeal membrane oxygenation was required in 19 patients (3.8%). The overall in-hospital mortality was 4.8% and was significantly higher in neonates and infants compared with older children (10% vs 11.8% vs 3.2%, P = .003). The length of hospital stay was longer in the neonatal group (median, 57 days, interquartile range, [24.8-90] vs 29.5 days [15.5-61] vs 10 days [7-18], P < .01). Nonhome discharges were more common in neonates and infants (40% vs 36.8% vs 13.1%, P < .01). Conclusion: Mechanical mitral valve replacement is increasingly performed over time with acceptable in-hospital morbidity and mortality, especially in older children and adolescents. Neonates and infants are associated with worse hospital survival, prolonged hospitalization, and significant rates of nonhome discharges.

Surgical Considerations for the Mitral Valve in Congenitally Corrected Transposition.

BACKGROUND: Systemic atrioventricular valve (morphologic tricuspid valve [TV]) regurgitation has been implicated in the development of systemic ventricular failure in congenitally corrected transposition of the great arteries (ccTGA), leading to timely referral for valve replacement. However, the surgical management of subpulmonary atrioventricular valve (morphologic mitral valve [MV]) regurgitation and outcomes has not been well studied. METHODS: Of 108 ccTGA patients undergoing atrioventricular valve surgery from 1979 to 2022, 22 patients (20%) underwent MV surgery. Demographics, etiology of valve regurgitation, operative details, and outcomes of these 22 patients were retrospectively reviewed. Follow-up at 1, 5, and 10 years was available for 18 (82%), 13 (59%), and 11 patients (50%), respectively. RESULTS: Median age was 37 years (interquartile range, 29-57 years). Intrinsic MV pathology was present in 18 individuals (82%). Operations included repair in 16 patients (73%), replacement in 6 (27%), and concomitant replacement of TV in 16 (73%). There was 1 perioperative death (5%) in a patient undergoing an emergent operation for severe acute-on-chronic heart failure due to worsening TV regurgitation. During a median follow-up of 12 years (interquartile range, 2-19 years), 7 patients (32%) died. Among the 16 patients who underwent repair, recurrent moderate or greater regurgitation was seen in 15%, 29%, and 43% of repairs in patients with annular, leaflet, and lead-induced regurgitation, respectively. CONCLUSIONS: Concomitant TV and MV disease occur much less frequently than isolated TV disease in ccTGA. Intrinsic MV disease is most commonly observed but appears less amenable to successful repair compared with mitral repair in the systemic position and suggests MV replacement may be preferred in ccTGA patients.