World Journal for Pediatric and Congenital Heart Surgery

Supravalvar aortic stenosis (SVAS) is a rare congenital anomaly. The “single-patch technique,” “‘two sinus augmentation with an inverted Y-patch” (both nonsymmetrical corrections), “three-patch technique,” and the “slide aortoplasty” (both symmetrical corrections) are the techniques implemented by the majority of surgeons for the correction of SVAS. In the few studies that compared these techniques, no technique was shown to be superior over another. The aim of the present study is to review the 52-year experience with the surgical correction of SVAS in two of four congenital cardiothoracic surgical centers in the Netherlands.

We retrospectively reviewed all patient files of those who underwent an operation to correct their SVAS, between 1962 and 2014 in our centers. Patients were divided according to their operating technique. These groups were compared using the end points freedom from reoperation and mortality.

A total of 49 patients were included, 23 (46.9%) patients in the nonsymmetrical group and 26 (53.1%) patients in the symmetrical group. Survival after 20 years in the nonsymmetrical group was 80% (standard error [SE]: 0.091) and in the symmetrical group was 85% (SE: 0.085; P = .163). Freedom from reoperation after 20 years in the nonsymmetrical group was 88% (SE: 0.079) and in the symmetrical group was 71% (SE: 0.107; P = 0.313).

In this patient group, there is no significant difference in survival and freedom from reoperation between the different surgical techniques for SVAS repair. Compared to the survival in the general population, the survival of SVAS patients is remarkably low. Apparently, SVAS is not a benign disease and probably patients should be followed more closely for the rest of their lives.

Controversy remains regarding the optimal surgical approach for children with supravalvular aortic stenosis (SVAS).

Since 1997 we have used Brom three-patch aortoplasty for patients with SVAS. We prefer computed tomography (CT) imaging for preoperative evaluation rather than cardiac catheterization as it avoids the well-known morbidity of general anesthesia. The purpose of this study was to present our intermediate-term results of this strategy.

Twenty consecutive patients with SVAS were treated with Brom aortoplasty. Mean age was 3.7 ± 5.9 years (median, 1.5 years). Twelve patients had Williams syndrome. Ten patients had preoperative advanced medical imaging (seven CT, three magnetic resonance imaging) and did not have cardiac catheterization. Mean times for cardiopulmonary bypass and cross-clamp were 172 ± 29 minutes and 110 ± 21 minutes, respectively. Ten patients had simultaneous pulmonary artery stenosis patching. Median length of stay was seven days. There was no operative or late mortality. Mean follow-up was 6 ± 5 years. There were no reoperations on the aortic root. Fifteen patients had mild or less aortic insufficiency (AI) and two had moderate AI. One patient who had infant balloon dilation of the aortic valve and postoperative subacute bacterial endocarditis had moderate-to-severe AI and aortic stenosis (AS). One patient had moderate residual SVAS; all others had no AS. No patients had late coronary insufficiency.

Brom aortoplasty promotes restoration of normal aortic root geometry and relief of coronary ostial stenosis, which is important in preventing myocardial ischemia. Computed tomography imaging is our preferred diagnostic modality. Intermediate-term outcomes are excellent with no recurrent SVAS, coronary events, or reoperations on the aortic valve.

Japan Congenital Cardiovascluar Surgical Database (JCCVSD) is a nationwide registry whose data are used for health quality assessment and clinical research in Japan. We evaluated the completeness of case registration and the accuracy of recorded data components including postprocedural mortality and complications in the database via on-site data adjudication.

We validated the records from JCCVSD 2010 to 2012 containing congenital cardiovascular surgery data performed in 111 facilities throughout Japan. We randomly chose nine facilities for site visit by the auditor team and conducted on-site data adjudication. We assessed whether the records in JCCVSD matched the data in the source materials.

We identified 1,928 cases of eligible surgeries performed at the facilities, of which 1,910 were registered (99.1% completeness), with 6 cases of duplication and 1 inappropriate case registration. Data components including gender, age, and surgery time (hours) were highly accurate with 98% to 100% concordance. Mortality at discharge and at 30 and 90 postoperative days was 100% accurate. Among the five complications studied, reoperation was the most frequently observed, with 16 and 21 cases recorded in the database and source materials, respectively, having a sensitivity of 0.67 and a specificity of 0.99.

Validation of JCCVSD database showed high registration completeness and high accuracy especially in the categorical data components. Adjudicated mortality was 100% accurate. While limited in numbers, the recorded cases of postoperative complications all had high specificities but had lower sensitivity (0.67-1.00). Continued activities for data quality improvement and assessment are necessary for optimizing the utility of these registries.

First-stage single-ventricle palliation is challenging to manage, and significant interstage morbidity and mortality remain. Prior computational and in vitro studies of the assisted bidirectional Glenn (ABG), a novel first-stage procedure that has shown potential for early conversion to a more stable augmented Glenn physiology, demonstrated increased pulmonary flow and oxygen delivery while decreasing cardiac work, as compared to conventional stage-1 alternatives. This study aims to identify optimal shunt designs for the ABG to improve pulmonary flow while maintaining or decreasing superior vena caval (SVC) pressure.

A representative three-dimensional model of a neonatal bidirectional Glenn (BDG) was created, with a shunt connecting the innominate artery to the SVC. The shunt design was studied as a six-parameter constrained shape optimization problem. We simulated hemodynamics for each candidate designs using a multiscale finite element flow solver and compared performance against designs with taper-less shunts, the standalone BDG, and a simplified control volume model. Three values of pulmonary vascular resistance (PVR) of 2.3, 4.3, and 7.1 WUm² were studied.

Increases in pulmonary flow were generally accompanied by increases in SVC pressure, except at low PVR (2.3 WUm²), where the optimal shunt geometry achieved a 13% increase in pulmonary flow without incurring any increase in SVC pressure. Shunt outlet area was the most influential design parameter, while others had minimal effect.

Assisted bidirectional Glenn performance is sensitive to PVR and shunt outlet diameter. An increase in pulmonary flow without a corresponding increase in SVC pressure is possible only when PVR is low.

Quantifying right ventricular function in patients with a systemic right ventricle (RV) is difficult but important for prognosis. Tissue motion annular displacement tracks displacement of the tricuspid annulus toward the apex. We evaluated this measure alongside fractional area change (FAC) on patients with single, RV prior to the bidirectional Glenn procedure. We tested both measures for correlation with outcomes.

Retrospective measurement of tissue motion annular displacement and FAC was performed on echocardiographic clips obtained prior to the bidirectional Glenn. A chart review included postoperative outcomes and midterm mortality/transplant. Bivariate correlations and Cox proportional hazards models were used for analyses.

Fifty-one patients with dominant RV underwent the bidirectional Glenn procedure and all had image quality that allowed analysis. The age ranged from 3 to 11 months (median 4 months). Neither tissue motion annular displacement nor FAC correlated with short-term postoperative outcomes. Tissue motion annular displacement was independently predictive of mortality/transplant (P = .03) in the Cox hazard model. The mean for survivors was 12.4% and for nonsurvivors/transplants was 10.0%. Tissue motion annular displacement intra-observer variability was 2.8% (1.2%-3.5%). Interobserver mean variability was 6.1% (3.3%-8.1%). Fractional area change was not predictive of mortality/transplant.

Tissue motion annular displacement is an independent predictor of midterm mortality/transplant after the bidirectional Glenn procedure in patients with single, RV, in this study. It may outperform FAC in this regard and has good reproducibility. Tissue motion annular displacement may be a useful measure in identifying high-risk children in this population.

Obesity is associated with increased lifelong morbidity and reduced life span and is increasingly prevalent in the congenital heart disease population. Habitual exercise is an important aspect of a healthy lifestyle and primary prevention of obesity in the general population. The association between habitual activity and body mass index (BMI) has not been studied in children with congenital heart disease.

A cross-sectional analysis of two previously collected cohorts was performed, including participants 8 to 18 years old with tetralogy of Fallot, transposition of the great arteries, and single ventricle heart disease after a Fontan operation. The association between BMI and duration of habitual exercise (measured by questionnaire) was studied. Secondary analyses assessing the effect of other possible factors for BMI were performed.

In total, 172 participants were studied (45% Tetralogy of Fallot, 12% transposition of the great arteries, and 43% Fontan). Median BMI was 18.2, and 29% of the participants were obese or overweight. Median habitual exercise was 5.9 h/wk. Thirty-eight percent of participants reported having their activity restricted by their cardiologist. Increasing exercise duration was associated with lower BMI (P = .01) in univariate analysis. In secondary analyses, restriction to mild exertion and participation in low-intensity exercise were both associated with increased BMI.

Increased habitual activity was associated with lower BMI, emphasizing the potential role of recreational sport in the health of children with congenital heart disease.

Cardiac rehabilitation (CR) improves exercise capacity and quality of life while reducing mortality in adults with acquired heart disease. Cardiac rehabilitation has not been extensively studied in adults with congenital heart disease (CHD).

We performed a prospective, randomized controlled trial (NCT01822769) of a 12-week clinical CR program compared with standard of care (SOC). Participants were ≥16 years old, had moderate or severe CHD, had O₂ saturation ≥92%, and had peak O₂ consumption ( V ̇ O 2 pk ) < 80% predicted. We assessed exercise capacity, physical activity, quality of life, self-reported health status, and other variables at baseline and after 12 weeks. The prespecified primary end point was change in V ̇ O 2 pk .

We analyzed data on 28 participants (aged 41.1 ± 12.1 years, 50% male), 13 randomized to CR and 15 to SOC. V ̇ O 2 pk averaged 16.8 ± 3.8 mL/kg/min, peak work rate = 95 ± 28 W, and median Minnesota Living with Heart Failure Questionnaire (MLHFQ) score = 27 (interquartile range: 11-44). Cardiac rehabilitation participants were older (48 ± 9 years vs 36 ± 12 years; P = .01), but there were no significant between-group differences in other variables. There were no adverse events related to CR. V ̇ O 2 pk increased in the CR group compared with SOC (+2.2 mL/kg/min, 95% confidence interval: 0.7-3.7; P = .002, age-adjusted +2.7 mL/kg/min; P = .004); there was a nonsignificant improvement in work rate (+8.1 W; P = .13). Among the 25 participants with baseline MLHFQ > 5, there was a clinically important >5-point improvement in 72.7% and 28.6% of CR and SOC participants, respectively (P = .047). Cardiac rehabilitation was also associated with improved self-assessment of overall health (P < .04).

Cardiac rehabilitation is safe and is associated with improvement in aerobic capacity and self-reported health status compared with SOC in adults with CHD.

Arterial switch operation (ASO) is a relatively safe operation nowadays. We hypothesize that intraoperative revision for coronary malperfusion still plays an important role during ASO in the current era.

From January 2005 to May 2016, 244 patients underwent ASO. Medical records were reviewed and the details of intraoperative revision were described. Morbidity and mortality were compared in patients with/without intraoperative revision. Factors related to the need for intraoperative revision were analyzed.

Seventeen (7%) patients needed intraoperative revision. As a technique for intraoperative revision, revision of a coronary button was performed in eight patients and revision of the main pulmonary artery anastomosis to relieve compression on the coronary artery was performed in five patients. Factors related to intraoperative revision were body weight at surgery (P = .051), eccentric position of the coronary ostium (P = .01), single coronary artery system (P = .03), and intramural coronary artery (P = .003). The commonest coronary artery origin and branching pattern was not protective against the need for intraoperative revision (P = .43). Discharge mortality was 2% overall: 2 of 17 with intraoperative revision versus 3 of 238 without it (P = .04). Patients who underwent intraoperative revision had longer postoperative hospitalization (P = .003).

The need for intraoperative revision was related to eccentric coronary ostium, single coronary artery, and intramural coronary artery. Although the need for intraoperative revision correlated with higher mortality and morbidity, prompt intraoperative revision also likely contributed to our good results after ASO.

Although pulmonary artery banding (PAB) is a common palliative procedure for pediatric heart malformation, there are concerns of pressure overload and concomitant immune reactions in the right ventricle causing postsurgical complications such as pericardial effusion. At this time, no clear guidelines as to potential risk factors or procedural contraindications have been widely disseminated. Therefore, a study was undertaken to examine wide-ranging factors to find potential biomarkers for postsurgical pericardial effusion formation risk.

A retrospective study was conducted on all cardiac surgeries performed over an eight-year period, and the main inclusion criterion was pericardial effusion development after PAB that required surgical drainage. Nine cases were then analyzed against a control group of 45 cases with respect to body measurements, concomitant surgeries, genetic screens, laboratory tests results, and cardiac function parameters.

Trisomy 21 was strongly associated with the development of severe pericardial effusion after PAB, and postoperative serum albumin levels in patients with trisomy 21 were associated with pericardial effusion development. Other parameters showed no significant correlation with pericardial effusion development.

Our data indicate a strong association between trisomy 21 and pericardial effusion requiring drainage after PAB, which is in line with translational research findings. Pressure overload from PAB may play a role in the formation of severe pericardial effusion that is exacerbated by cardiac structural defects commonly associated with trisomy 21. Surgical teams should therefore use caution and plan to implement drainage in PAB cases, and postoperative serum albumin may serve as a useful biomarker for pericardial effusion formation.

Postoperative care delivered in the pediatric cardiac intensive care unit (CICU) relies on providers’ understanding of patients’ congenital heart defects (CHDs) and procedure performed. Novel, bedside use of virtual, three-dimensional (3D) heart models creates access to patients’ CHD to improve understanding. This study evaluates the impact of patient-specific virtual 3D heart models on CICU provider attitudes and care delivery.

Virtual 3D heart models were created from standard preoperative cardiac imaging of ten patients with CHD undergoing repair and displayed on a bedside tablet in the CICU. Providers completed a Likert questionnaire evaluating the models’ value in understanding anatomy and improving care delivery. Responses were compared using two-tailed t test and Mann-Whitney U test and were also compared to previously collected CICU provider responses regarding use of printed 3D heart models.

Fifty-three clinicians (19 physicians, 34 nurses/trainees) participated; 49 (92%) of 53 and 44 (83%) of 53 reported at least moderate to high satisfaction with the virtual 3D heart’s ability to enhance understanding of anatomy and surgical repair, respectively. Seventy-one percent of participants felt strongly that virtual 3D models improved their ability to manage postoperative problems. The majority of both groups (63% physicians, 53% nurses) felt that virtual 3D heart models improved CICU handoffs. Virtual 3D heart models were as effective as printed models in improving understanding and care delivery, with a noted provider preference for printed 3D heart models.

Virtual 3D heart models depicting patient-specific CHDs are perceived to improve understanding and postoperative care delivery in the CICU.