Adults with Congenital Heart Disease and Transplant: Challenges, Opportunities, and Policy.

The rate of heart transplantation in adults with congenital heart disease (ACHD) is rising, but the 1-year mortality posttransplantation remains higher than non-ACHD patients. A robust pretransplant assessment and operative and postoperative planning can mitigate much of the perioperative risk. Importantly, ACHD patients who survive the first year have significantly better 10-year survival compared with non-ACHD patients. The current allocation system gives ACHD patients a relatively high priority, but providers must use the prespecified exception requests for higher status, especially for patients with the Fontan circulation. It is vital that ACHD patients with end-stage heart failure are cared for at centers with ACHD subspecialty care.

Pulmonary Atresia With Intact Ventricular Septum: From Radiofrequency Perforation to Transcatheter Pulmonary Valve.

OBJECTIVES: We intend to describe an entirely transcatheter management pathway for patients with pulmonary atresia with intact ventricular septum (PA/IVS). BACKGROUND: PA/IVS is a rare cyanotic congenital heart lesion traditionally palliated with multiple procedures and surgeries. Entirely non-surgical transcatheter management from infancy to adulthood may be possible; however, the pathway from neonatal radiofrequency (RF) pulmonary valve perforation to later transcatheter pulmonary valve replacement (TPV) is not well described. METHODS: This retrospective study was performed at a pediatric cardiac center between 2007-2018. All patients with PA/IVS who were managed exclusively with catheterization-based interventions as neonates (RF perforation, pulmonary valvuloplasty, or ductal stenting) were analyzed. Demographic, procedural and clinical data were collected. RESULTS: Fifteen patients had exclusively catheterization-based RV decompression as neonates, 7 of whom did not require subsequent surgery. Six patients required a right ventricular outflow tract (RVOT) augmentation later in life; all were born before 2013. All 6 later had a TPV placed. Two of the patients underwent a surgical Glenn shunt alone. Of the 7 patients that never had surgery, 3 have since undergone a TPV, and 4 are awaiting candidacy for TPV placement. No patients with PA/IVS have had an RVOT augmentation at our institution since 2012. CONCLUSIONS: Patients with PA/IVS who undergo catheterization-based neonatal interventions can make it to TPV without requiring surgery. At our institution, there has been a shift in management over the last 8 years favoring entirely non-surgical transcatheter management from infancy to adulthood.

Laparoscopic Surgery Requiring Abdominal Insufflation in Patients With Congenital Heart Disease.

OBJECTIVE: To determine if patients with congenital heart disease are undergoing laparoscopic surgery requiring abdominal insufflation and to compare the outcomes of these procedures with those who underwent an open surgical approach. DESIGN, SETTING, PARTICIPANTS: This was a retrospective study using the National Inpatient Sample from 2006 to 2014. Individuals with congenital heart disease who underwent at least one of six selected surgical procedures (laparoscopic or open) were included in the study. Subgroup analysis was performed on patients with Fontan palliation. MEASUREMENTS AND MAIN RESULTS: The primary outcome was to determine the frequency with which congenital heart disease patients undergo laparoscopic surgery requiring abdominal insufflation compared with open surgery. Secondary outcomes included all-cause in-hospital mortality and in-hospital length of stay. Of the 5,527 patients included, nearly half underwent laparoscopic surgery (46.3%), and 128 (2.3%) had single-ventricle circulation. All-cause mortality was significantly higher for those who underwent open surgery compared with the laparoscopic approach (3.6% v 0.9%; odds ratio [OR], 4.0 [2.6-6.3]; p < 0.0001). Subgroup analysis of patients with Fontal palliation older than five years showed 30 (42%) underwent laparoscopic surgery and there was no mortality difference between the laparoscopic and open approaches (OR, 1.4 [0.2-21.3], p = 0.8). Length of stay was significantly shorter for patients undergoing laparoscopic compared with open surgery (median three days [interquartile range, two-five] v six days [three-13], p < 0.0001). CONCLUSIONS: Individuals with congenital heart disease are being offered laparoscopic surgery that requires abdominal insufflation. All-cause mortality and length of stay were higher for patients who underwent open surgical operations.

Priorities and Understanding of Pregnancy Among Women With Congenital Heart Disease: A Mixed-Methods Study.

Background: Many women with congenital heart disease (CHD) desire safe and successful pregnancies, but a significant proportion does not seek prepregnancy counseling. Objectives: This study aims to distinguish the personal priorities and perceptions about pregnancy in this growing population. Methods: Women aged 18 to 50 years with CHD were enrolled from 2 sites. Using a mixed-methods approach (Q-methodology), 179 participants sorted 23 statements representing a collection of views on pregnancy using priority forced ranking along a scale from "strongly agree" to "strongly disagree." Results: Majority of women were between 25 and 29 years of age, had moderate or severely complex CHD, and were married. Five unique group identities were elucidated from patient responses. Group 1 was centered around a strong desire to start a family. Women in group 2 had significant anxiety, and their psychological wellbeing interfered with their decision to start a family. Women in group 3 were concerned about premature death; if they do have kids, they want to be alive to see them grow old. Women in group 4 had strong objections to termination. Group 5 valued health care engagement. Group identities were unrelated to CHD complexity and demographic factors such as age and marital status. Six differentiating statements were identified that help distinguish which group a woman aligns with. Conclusions: Women with CHD have diverse priorities and values relating to pregnancy and heart disease. This study used a mixed-methods approach to provide a framework identifying several domains for targeted prepregnancy counseling in women with CHD.

Infants with pulmonary atresia intact ventricular septum who require balloon atrial septostomy have significantly higher 18-month mortality.

INTRODUCTION: Many newborns with pulmonary atresia/intact ventricular septum require intervention to establish pulmonary flow and sufficient cardiac output. The resulting haemodynamic changes are not well characterised and may have unintended consequences. METHODS: This is a 30-year (1988-2018) retrospective study of patients with pulmonary atresia intact ventricular septum. RESULTS: Eighty-nine patients were included, and median follow-up was 8 years. Fifty-five per cent had coronary sinusoids and 27% had right ventricular-dependent coronary circulation. Most patients were managed with surgical aortopulmonary or modified Blalock-Taussig shunt (73%), and 12 patients underwent balloon atrial septostomy before surgical intervention. The remaining patients (27%) underwent only transcatheter interventions; 7 required an atrial septostomy and 17 required ductal stentings. All-cause mortality was 10%, most deaths (89%) occurred before 18 months of age. Of these early deaths, 87% required a balloon atrial septostomy and 85% had right ventricular-dependent coronary sinusoids. Eighteen-month mortality was significantly higher for patients who required a balloon atrial septostomy compared to those who did not (36% versus 1.4% p < 0.0001). DISCUSSION: Patients with pulmonary atresia/intact ventricular septum who require balloon atrial septostomy in the newborn period have significantly higher 18-month mortality. Quantifying the mortality difference may help guide prognostication and expectation setting. Infants who had septostomy and a surgical shunt in the newborn period fared better than those who only underwent septostomy (even when accompanied by ductal stenting). For infants with right ventricular-dependent circulation, atrial septostomy should only be performed on an urgent or emergent basis and these patients should be considered for early surgical intervention and neonatal transplant.

Antiarrhythmic Treatment Duration and Tachycardia Recurrence in Infants with Supraventricular Tachycardia.

We sought to assess the effect of a shorter medication treatment course (up to 4-6 months of age) on the recurrence of infantile supraventricular tachycardia (SVT). This was a retrospective review of infants with SVT diagnosed at age 0-12 months at Rady Children's Hospital (2010-2017). Infants with structural congenital heart disease, automatic tachycardias, atrial flutter, or lack of follow-up data were excluded. Seventy-four infants met criteria. Median age at diagnosis was 6 days (IQR 0-21 days); 28.4% presented with fetal tachycardia. Median gestational age was 38.4 weeks (IQR 36-40), 30% were preterm. Median age at medication discontinuation was 6.7 months (IQR 4.6-9.8). Therapy was stopped at younger age in patients managed by pediatric electrophysiologist (vs. general pediatric cardiologist): 4.9 vs. 8.6 months (p = 0.03). Thirty-eight patients (51.4%) were treated for < 6 months; 32.4% for 6-12 months, and 16.2% for > 12 months. SVT recurrence was similar for these groups: 13.2% vs. 16.7%, and 33.3%, respectively, (p = 0.27). Most patients with recurrence required emergency care, though none had significant adverse outcomes. Infants with SVT and structurally normal cardiac anatomy, who remain recurrence free on a single agent, have no increased risk of recurrence with shorter treatment courses of 4-6 months, compared to traditional treatment duration of 6-12 months.

Successful Management of a Patient With Jacobsen Syndrome and Hypoplastic Left Heart Syndrome.

Jacobsen syndrome (JS) is a rare genetic condition characterized by intellectual disability, hematologic abnormalities, and congenital heart defects. A male infant presented at birth with phenotypic findings of JS and echocardiographic findings of hypoplastic left heart syndrome (HLHS). Array comparative genomic hybridization was performed at age three days and revealed an 8.1 Mb terminal deletion on the long arm of chromosome 11, consistent with JS. At five days of age, a hybrid stage 1 procedure was performed. At age 46 days, he underwent a Norwood operation followed by bidirectional Glenn at age six months. He is presently 23 months old and doing well. With careful consideration of the individual patient and comorbidities associated with JS, we propose that at least a subset of patients with JS and HLHS can do well with staged surgical palliation.

Arterial Switch Operation in a Patient With Ehlers-Danlos Syndrome Type IV.

Ehlers-Danlos syndrome vascular type IV is characterized by translucent skin, easy bruising, and fragility of arteries. A full-term female infant presented at four weeks of age with a diagnosis of d-transposition of the great arteries with restrictive atrial septal defect. She successfully underwent emergent balloon atrial septostomy and placement of patent ductus arteriosus (PDA) stent. She required restenting of the PDA and pulmonary artery banding prior to arterial switch procedure. At 16 months of age, the patient successfully underwent arterial switch procedure without complication. This report demonstrates the feasibility of an arterial switch operation along with long-term follow-up of this rare condition.

Aortopulmonary Window With Pulmonary Atresia and Interrupted Aortic Arch: A Very Rare Triad.

Aortopulmonary window (APW) is a rare lesion, accounting for 0.2% to 0.6% of all congenital heart diseases. We report a rare case of an infant with APW, interrupted aortic arch, and pulmonary atresia with intact interventricular septum and right ventricle-dependent coronary circulation. This report describes the anatomy of this lesion set, the complex surgical palliation that was required, and the management of postoperative complications.

Process of Care in Breast Reconstruction and the Impact of a Dual-Trained Surgeon.

BACKGROUND: For postmastectomy reconstruction, the most common model in the United States is a two-team approach, consisting of breast and plastic surgeon. In other countries, a single-surgeon approach trained in both plastic and oncologic surgery is well described. We hypothesized that a dual-trained breast and plastic surgeon would decrease the postoperative care burden for the patient without compromising outcomes and serve as a model for team-centered breast reconstruction care. METHODS: A retrospective review was performed of patients undergoing mastectomy with immediate expander reconstruction from January 2013 to October 2014. Patient data up to 1 year postoperatively was recorded. Patients were stratified by treatment to "single-surgeon" or "two-surgeon" team. Demographic and operative data were recorded. Google Maps was used to calculate travel distance. A standard of mean cost of travel per mile and mean hourly wage for San Diego County was used. The primary outcome was the total number of postoperative clinic visits. In addition, factors predictive of postoperative clinic visits were evaluated. RESULTS: During the study period, 147 patients were included in analysis (69, single-surgeon; 78, two-surgeon). The mean cost of travel per mile was US $59.2 cents and mean hourly wage for San Diego County was US $25.49. For the 1-year follow-up period, patients with the single surgeon had a mean (SD) of 9.3 (3.72) postoperative visits compared with 15.6 (3.96) for patients in the two-surgeon team (P < 0.0001).There were no statistical differences between groups in the rate of complications. In the final model, treatment team, bilateral mastectomies, and complications (operative and nonoperative) were significant predictors of the total number of postoperative visits. Patients in the two-surgeon team spent an additional 11.13 hours and 216 miles commuting and in clinic. In total, the additional 6.3 clinic visits for patients in the two-surgeon team resulted in an average of US $695.33 additional dollars spent on travel and lost wages. CONCLUSIONS: Single-surgeon patients required fewer postoperative visits. Fewer postoperative clinic visits may have significant socioeconomic and psychological benefits to patients. Given these results, we believe that streamlining care into an integrated multidisciplinary model would be beneficial.

The Impact of Residents on Patient Satisfaction.

BACKGROUND: High-quality physician communication is the foundation for achieving high patient satisfaction. Increasing importance is placed on eliciting feedback from patients. However, there have been few studies looking at the impact of resident involvement on patient satisfaction. Our hospital system values the patient's likelihood to recommend the practice as the top marker for patient satisfaction. METHODS: Between May 2016 and December 2016 at University of California, San Diego, all outpatient appointments were randomly mailed Press-Ganey surveys or an eSurvey regarding their experience. The surveys were filtered based on resident participation, and an χ test was performed to assess the impact of residents. An additional aim was to determine the degree to which the impact of resident involvement differed between surgical specialties. Binomial probability was calculated for each specialty using the 'no resident' group as the reference percentage. RESULTS: A total of 73,834 surveys were mailed or sent electronically, and 17,653 surveys were returned (23.9% response rate). Overall, patients expressed high levels of satisfaction with the quality of physician communication. Patients who had residents involved in their care reported a decrease in satisfaction with physician communication and a decrease in the likelihood to recommend the practice (88.7% vs 90.4%, P < 0.001). In the analysis of resident impact by surgical specialty, 9 specialties qualified for analysis. Resident involvement was associated with lower physician communication scores in orthopedic surgery (P = 0.032), otolaryngology (P = 0.015), and vascular surgery (P = 0.01). In all other surgical subspecialties, there was no statistically significant difference between groups. CONCLUSIONS: Overall, patients expressed high levels of satisfaction with the quality of physician communication with and without resident involvement. Resident physician involvement in surgical clinic visits was associated with lower overall patient satisfaction and decreased likelihood of recommending the practice. In addition, we observed that resident involvement was not associated with lower communication scores in most surgical specialties, including Plastic Surgery.

Public unawareness of physician reimbursement.

OBJECTIVES: To assess subjects' perception of healthcare costs and physician reimbursement. BACKGROUND: The lack of transparency in healthcare reimbursement leaves patients and physicians unaware of the distribution of health care dollars. METHODS: Anonymous survey-based study by means of convenience sampling. Participants were asked to estimate the total hospital cost and physician fee for one of the six medical procedures (n = 250). RESULTS: On the average for all 6 procedures, patients estimated the total cost was $36,177, ∼1,540% more than the actual Medicare rate of $7,333. Similarly, patients estimated the physician fee was $7,694, 1,474% more the actual Medicare rate of $589. CONCLUSION: Patients' perception of the total cost and physician fee are significantly higher than Medicare rates for all 6 procedures. This lack of insight may have widespread negative implications on the patient-physician relationship, on political trends to reduce physician reimbursement, and on a physician's desire to continue practicing medicine.

Utility of Genetic Testing in Elite Volleyball Players with Aortic Root Dilation.

Basketball and volleyball attract individuals with a characteristic biophysical profile, mimicking features of Marfan syndrome. Consequently, identification of these abnormalities can be lifesaving. PURPOSE: To determine how physical examination, echocardiography, and genetic screening can identify elite volleyball players with a previously undiagnosed aortopathy. METHODS: We have performed cardiac screening on 90 US Volleyball National Team members and identified four individuals with dilated sinuses of Valsalva. This case series reports on three individuals who underwent a comprehensive genetics evaluation, including gene sequencing. RESULTS: Cardiac screening combined with genetic testing can identify previously undiagnosed tall athletes with an aortopathy, in the absence of noncardiac findings of a connective tissue disorder. Subject 1 had a revised Ghent systems (RGS) score of 2 and a normal aortopathy gene panel. Subject 2 had a RGS score of 1 and genetic testing revealed a de novo disease causing mutation in the gene encoding fibrillin-1 (FBN1). Subject 3 had an RGS score of 4.0 and had a normal aortopathy gene panel. CONCLUSIONS: Despite variable clinical features of Marfan syndrome, dilated sinuses of Valsalva were found in 4.9% of the athletes. A disease-causing mutation in the FBN1 gene was identified in subject 2, who had the lowest RGS but the largest aortic root measurement. Subjects 1 and 3, with the highest RGS, had a normal aortopathy gene panel. Our findings provide further evidence suggesting that a cardiac evaluation, including a screening echocardiogram, should be performed on all elite tall adult athletes independent of other physical findings. Genetic testing should be considered for athletes with dilated sinuses of Valsalva (male, >4.2 cm; female, >3.4 cm), regardless of other extracardiac findings.