Advances in Fetal Cardiac Imaging and Intervention.

The field of fetal cardiology has evolved significantly in recent years. This review focuses on specific advances in fetal cardiac imaging and intervention that are increasingly used in clinical practice. On the imaging frontier, updated screening guidelines and artificial intelligence hold promise for improving prenatal detection of congenital heart disease. Advances in ultrasound technology and magnetic resonance imaging techniques have enabled greater diagnostic and prognostic accuracy of fetal heart disease from the first to third trimesters, and maternal hyperoxygenation can offer additional physiological insights. Fetal cardiac therapy has also seen great progress, with advances in transplacental pharmacologic treatments, infusions of enzyme replacement therapy, and fetal surgery for select rare and severe conditions.

La cardiologie fœtale a connu un essor fulgurant ces dernières années. Nous nous penchons ici sur certaines avancées réalisées en imagerie et en thérapie cardiaque fœtale dont l'usage est de plus en plus répandu en pratique clinique. Du côté de l'imagerie, on se tourne vers les nouvelles recommandations sur le dépistage et vers l'intelligence artificielle pour améliorer le dépistage prénatal de la cardiopathie congénitale. Grâce aux avancées technologiques, l'échographie et l'IRM permettent de diagnostiquer une cardiopathie fœtale et d'établir le pronostic avec un plus haut degré de précision, et ce, du premier au troisième trimestre. L'hyperoxygénation maternelle, quant à elle, apporte un éclairage sur les effets physiologiques. La thérapie cardiaque fœtale a elle aussi fait des bonds de géant avec les traitements pharmacologiques transplacentaires, l'enzymothérapie de remplacement et la chirurgie fœtale pour certaines maladies rares et graves.

Repair of Aberrant Right Subclavian Artery Causing Dysphagia Lusoria via Partial Median Sternotomy.

While unusual, aberrant right subclavian artery (ARSCA) can occasionally be a source of significant dysphagia in children. We present a case of a 13-year-old female who reported a three-year history of dysphagia to solid foods and was found to have ARSCA on a barium swallow study and computed tomography scan of the chest. We reimplanted the ARSCA into the right carotid artery in end-to-side fashion using a partial median sternotomy approach. At six months follow-up, her symptoms had completely resolved, and her postoperative echocardiogram showed an unobstructed reimplanted ARSCA. Meeting presentation: AATS 102nd Annual Meeting; May 14, 2022; Boston, MA.

Peri-operative changes in diastolic function and outcomes in congenital aortic valve surgery.

BACKGROUND: The ratio of early diastolic mitral inflow velocity (E) to early diastolic mitral annular tissue velocity (e'), or E/e', is an echocardiographic measure of left ventricular filling pressure. Peri-operative changes in E/e' and association with outcomes have been demonstrated in adults undergoing surgery for aortic stenosis (AS). We sought to explore changes in E/e' and other diastolic indices in the setting of congenital AS surgery and to assess for association with post-operative outcomes among children and young adults. METHODS: A retrospective, single-center study was performed among patients 6 months to 30 years of age who underwent congenital AS surgery from 2006 to 2018. Tissue Doppler indices were collected from pre- and post-operative echocardiograms. Post-operative outcomes were reviewed. RESULTS: Sixty-six subjects with subvalvar (45%), valvar (47%), and supravalvar (8%) AS underwent surgery at a median age of 9.5 years (IQR: 4.0-14.8). Pre-operatively, the lateral E/e' ratio was 8.6 (6.7-11.0); 33% had E/e'≥10. Post-operatively, the lateral e' decreased to 9.9 cm/s (8.0-11.4), the E/e' ratio increased to 10.4 (8.3-13.1); and 53% had E/e'≥10 (p-values < 0.0001, 0.0072, and < 0.001, respectively). Pre-operative lateral e' correlated modestly with duration of intubation (ρ = -0.24, p-value 0.048) and post-operative lateral e' correlated modestly with duration of intubation and length of hospital stay (ρ = -0.28 and -0.26, p-values = 0.02 and 0.04, respectively). CONCLUSIONS: Children and young adults who underwent congenital AS surgery had echocardiographic evidence of diastolic dysfunction pre-operatively that worsened post-operatively. Lateral e' may be a sensitive indicator of impaired ventricular relaxation in these patients and may impact duration of intubation and hospital stay.

Weight impacts 1-year congenital heart surgery outcomes independent of race/ethnicity and payer.

Body mass index, race/ethnicity, and payer status are associated with operative mortality in congenital heart disease (CHD). Interactions between these predictors and impacts on longer term outcomes are less well understood. We studied the effect of body mass index, race/ethnicity, and payer on 1-year outcomes following elective CHD surgery and tested the degree to which race/ethnicity and payer explained the effects of body mass index. Patients aged 2-25 years who underwent elective CHD surgery at our centre from 2010 to 2017 were included. We assessed 1-year unplanned cardiac re-admissions, re-interventions, and mortality. Step-wise, multivariable logistic regression was performed.Of the 929 patients, 10.4% were underweight, 14.9% overweight, and 8.5% obese. Non-white race/ethnicity comprised 40.4% and public insurance 29.8%. Only 0.5% died prior to hospital discharge with one additional death in the first post-operative year. Amongst patients with continuous follow-up, unplanned re-admission and re-intervention rates were 14.7% and 12.3%, respectively. In multivariable analyses adjusting for surgical complexity and surgeon, obese, overweight, and underweight patients had higher odds of re-admission than normal-weight patients (OR 1.40, p = 0.026; OR 1.77, p < 0.001; OR 1.44, p = 0.008). Underweight patients had more than twice the odds of re-intervention compared with normal weight (OR 2.12, p < 0.001). These associations persisted after adjusting for race/ethnicity, payer, and surgeon.Pre-operative obese, overweight, and underweight body mass index were associated with unplanned re-admission and/or re-intervention 1-year following elective CHD surgery, even after accounting for race/ethnicity and payer status. Body mass index may be an important modifiable risk factor prior to CHD surgery.

Extracardiac Doppler indices predict perinatal mortality in fetuses with Ebstein anomaly and tricuspid valve dysplasia.

OBJECTIVES: Ebstein anomaly and tricuspid valve dysplasia (EA/TVD) carry high perinatal mortality. Past studies have focused on cardiac predictors of mortality; we sought to describe the fetal echo (FE) extracardiac Dopplers in this cohort and determine their association with perinatal mortality. METHOD: Fetuses with EA/TVD at 23 centers from 2005-2011 were included for retrospective study. Doppler pattern and velocity of the umbilical artery (UA), umbilical vein (UV), ductus venosus (DV), and middle cerebral artery (MCA) were collected. Bivariate and multivariate analyzes were performed. The primary outcome measure was perinatal mortality, defined as fetal demise or neonatal death. RESULTS: Of 190 cases that met eligibility criteria, alterations were seen in 50% of UA, 16% of UV, 48% of DV, and 8% of MCA Doppler indices on the last FE (median 27.4 weeks). Independent predictors of perinatal mortality included abnormal UA Doppler pattern of absence or reversed end diastolic flow (OR 9.7) and UV velocity z score <1 (OR 2.5), in addition to diagnosis <32 weeks (OR 4.2) and tricuspid valve (TV) annulus z score ≥6 (OR 5.3). CONCLUSION: Abnormal UA Doppler pattern and decreased UV velocity are independent predictors of perinatal mortality in EA/TVD fetuses and should be used to refine mortality risk and guide perinatal management.

Effect of In Utero Non-Steroidal Anti-Inflammatory Drug Therapy for Severe Ebstein Anomaly or Tricuspid Valve Dysplasia (NSAID Therapy for Fetal Ebstein anomaly).

Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) are rare congenital malformations associated with nearly 50% mortality when diagnosed in utero. The diseases often produce severe tricuspid regurgitation (TR) in the fetus and in some cases, pulmonary regurgitation (PR) and circular shunting ensue. Since the ductus arteriosus (DA) plays a critical role in the circular shunt and may be constricted by transplacental nonsteroidal anti-inflammatory drugs (NSAIDs), we sought to assess the effect of NSAIDs on fetuses with EA/TVD. We reviewed mothers of singleton fetuses with EA/TVD and PR, indicative of circular shunting, who were offered NSAIDs at multiple centers from 2010 to 2018. Initial dosing consisted of indomethacin, followed by ibuprofen in most cases. Twenty-one patients at 10 centers were offered therapy at a median gestational age (GA) of 30.0 weeks (range: 20.9 to 34.9). Most (15/21 = 71%) mothers received NSAIDs, and 12 of 15 (80%) achieved DA constriction after a median of 2.0 days (1.0 to 6.0). All fetuses with DA constriction had improved PR; 92% had improved Doppler patterns. Median GA at pregnancy outcome (live-birth or fetal demise) was 36.1 weeks (30.7 to 39.0) in fetuses with DA constriction versus 33 weeks (23.3 to 37.3) in fetuses who did not receive NSAIDs or achieve DA constriction (p = 0.040). Eleven of 12 patients (92%) with DA constriction survived to live-birth, whereas 4 of 9 patients (44%) who did not receive NSAIDs or achieve DA constriction survived (p = 0.046). In conclusion, our findings demonstrate the proof of concept that NSAIDs mitigate circular shunt physiology by DA constriction and improve PR among fetuses with severe EA/TVD. Although the early results are encouraging, further investigation is necessary to determine safety and efficacy.

Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study.

Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. Methods and Results This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia. Among live-born patients, clinical and echocardiographic factors were evaluated for association with neonatal mortality and palliated versus biventricular circulation at discharge. Of 176 live-born patients, 7 received comfort care, 11 died <24 hours after birth, and 4 had insufficient data. Among 154 remaining patients, 38 (25%) did not survive to discharge. Nearly half (46%) underwent intervention. Mortality differed by procedure; no deaths occurred in patients who underwent right ventricular exclusion. At discharge, 56% of the cohort had a biventricular circulation (13% following intervention) and 19% were palliated. Lower tricuspid regurgitation jet velocity (odds ratio [OR], 2.3 [1.1-5.0], 95% CI, per m/s; P=0.025) and lack of antegrade flow across the pulmonary valve (OR, 4.5 [1.3-14.2]; P=0.015) were associated with neonatal mortality by multivariable logistic regression. These variables, along with smaller pulmonary valve dimension, were also associated with a palliated outcome. Conclusions Among neonates with Ebstein anomaly or tricuspid valve dysplasia diagnosed in utero, a variety of management strategies were used across centers, with poor outcomes overall. High-risk patients with low tricuspid regurgitation jet velocity and no antegrade pulmonary blood flow should be considered for right ventricular exclusion to optimize their chance of survival.

Outcomes of Surgical Repair of Complex D-Transposition of the Great Arteries.

BACKGROUND: D-transposition of the great arteries (TGA) or TGA-type double outlet right ventricle (DORV) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) may be treated with the arterial switch operation (ASO), Rastelli, Réparation à l'Etage Ventriculaire (REV), or Nikaidoh procedures. We evaluated midterm results of these techniques. METHODS: We retrospectively reviewed 42 cases of anatomic repair from 2005 to 2014 at our institution for TGA (n = 29) or TGA-type DORV (n = 13) with VSD and LVOTO. We analyzed outcomes (mortality, reoperation, residual/recurrent LVOT peak gradient ≥20 mm Hg, right ventricular outflow tract [RVOT] peak gradient ≥40 mm Hg) and performed a risk analysis. Mean follow-up was 5.77 ± 3.08 years. RESULTS: Seventeen (40.5%) patients had an ASO with (n = 4) or without (n = 13) LVOTO resection. The Rastelli, REV, and Nikaidoh procedures were used in 14 (33%), 5 (12%), and 6 (14%) patients, respectively. There were no mortalities or moderate aortic insufficiency at last follow-up. Three (9%) patients developed LVOT gradient ≥20 mm Hg, while nine (21.4%) patients had RVOT obstruction. Reoperations included RVOT/pulmonary artery reoperation (n = 10; 23.8%) and LVOT reoperation (n = 1; 2.5%). Freedom from reoperation was 84% ± 6% and 75% ± 9% at one and three years, respectively, for the entire cohort with no differences between groups by type of operation (log-rank P = .64). The Nikaidoh procedure compared favorably to all other techniques in terms of reoperation (n = 0/6; 0% vs n = 13/36; 36%; P = .08). CONCLUSIONS: Midterm outcomes after anatomic repair for TGA or TGA-type DORV with LVOTO and VSD are excellent but vary by surgical technique. The Nikaidoh procedure seems to compare favorably to the other techniques.

Significance of Intraoperative Revision During Arterial Switch Operation in the Current Era.

BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

Assessment of the Melody valve in the mitral position in young children by echocardiography.

OBJECTIVES: Mitral valve replacement (MVR) in young children is limited by the lack of small prostheses. Our institution began performing MVR with modified, surgically placed, stented jugular vein grafts (Melody valve) in 2010. We sought to describe key echocardiographic features for pre- and postoperative assessment of this novel form of MVR. METHODS: The pre- and postoperative echocardiograms of 24 patients who underwent Melody MVR were reviewed. In addition to standard measurements, preoperative potential measurements of the mitral annulus were performed whereby dimensions were estimated for Melody sizing. A ratio of the narrowest subaortic region in systole to the actual mitral valve dimension (SubA:MV) was assessed for risk of postoperative left ventricular outflow tract obstruction (LVOTO). RESULTS: Melody MVR was performed at a median of 8.5 months (5.6 kg) for stenosis (5), regurgitation (3), and mixed disease (16). Preoperatively, actual mitral z scores measured hypoplastic (median -3.1 for the lateral [lat] dimension; -2.1 for the anteroposterior [AP] dimension). The potential measurements often had normal z scores with fair correlation with intraoperative Melody dilation (ρ = 0.51 and 0.50 for lat and AP dimensions, respectively, both P = .01). A preoperative SubA:MV <0.5 was associated with postoperative LVOTO, which occurred in 4 patients. Postoperatively, mitral gradients substantially improved, with low values relative to the effective orifice area of the Melody valve. No patients had significant regurgitation or perivalvar leak. CONCLUSIONS: Preoperative echocardiographic measurements may help guide intraoperative sizing for Melody MVR and identify patients at risk for postoperative LVOTO. Acute postoperative hemodynamic results were favorable; however, ongoing assessment is warranted.

Assessment of Progressive Pathophysiology After Early Prenatal Diagnosis of the Ebstein Anomaly or Tricuspid Valve Dysplasia.

In fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD), poor hemodynamic status is associated with worse neonatal outcome. It is not known whether EA/TVD fetuses with more favorable physiology earlier in gestation progress to more severe disease in the third trimester. We evaluated if echocardiographic indexes in EA/TVD fetuses presenting <24 weeks of gestation are reliable indicators of physiologic status later in pregnancy. This multicenter, retrospective study included 51 fetuses presenting at <24 weeks of gestation with EA/TVD and serial fetal echocardiograms ≥4 weeks apart. We designated the following as markers of poor outcome: absence of anterograde flow across the pulmonary valve, pulmonary valve regurgitation, cardiothoracic area ratio >0.48, left ventricular (LV) dysfunction, or tricuspid valve (TV) annulus Z-score >5.6. Median gestational age at diagnosis was 21 weeks (range, 18 to 24). Eighteen fetuses (35%) had no markers for poor hemodynamic status initially, whereas only 7 of these continued to have no markers of poor outcome in the third trimester. Nine of 27 fetuses (33%) with anterograde pulmonary blood flow on the first echocardiogram developed pulmonary atresia; 7 of 39 (18%) developed new pulmonary valve regurgitation. LV dysfunction was present in 2 (4%) patients at <24 weeks but in 14 (37%) later (p <0.001). The TV annulus Z-score and cardiothoracic area both increased from diagnosis to follow-up. In conclusion, progressive hemodynamic compromise was common in this cohort. Our study highlights that care must be taken in counseling before 24 weeks, as the absence of factors associated with poor outcome early in pregnancy may be falsely reassuring.

Growth and Obesity Among Older Single Ventricle Patients Presenting for Fontan Conversion.

INTRODUCTION: Long-term growth outcomes and the prevalence of obesity among older single ventricle (SV) patients have not been well characterized. We investigated these parameters, as well as the impact of obesity on survival, in an older cohort of SV patients presenting for Fontan conversion. METHODS: We analyzed preoperative height, weight, and body mass index (BMI) of patients who underwent Fontan conversion. Overweight and obese were defined as BMI ≥85 percentile and ≥95 percentile for patients <20 years and BMI 25 to 30 kg/m(2) and ≥30 kg/m(2) for patients ≥20 years, respectively. Postoperative transplant-free survival was assessed among obese, overweight, and normal weight patients. RESULTS: We evaluated 139 patients presenting for Fontan conversion at a median age of 23.2 years. Patients had shorter stature compared to the normal population (mean Z score -0.6, P < .001). Younger patients had lower BMI compared to the normal population (<20 years: mean Z score -0.5, P = .02), while older patients had elevated BMI (≥20 years: mean Z score +0.4, P < .001). The mean BMI among older patients approached overweight at 24.6 kg/m(2). The prevalence of obesity increased with advancing age, with 36% overweight and 14% obese at >30 years. At a median of 8.2 years following Fontan conversion, obesity and overweight status were not associated with transplant-free survival. CONCLUSION: Older SV patients presenting for Fontan conversion had shorter stature compared to the normal population as well as a high prevalence of overweight and obesity. Although there was no relationship between weight status and early postoperative survival, further investigation of long-term outcomes is warranted.

Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study.

BACKGROUND: Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era. METHODS AND RESULTS: Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5-21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1-1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4-6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1-6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001). CONCLUSION: In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.

Left ventricular thrombus formation after repair of anomalous left coronary artery from the pulmonary artery.

Although thrombus formation following myocardial infarction in adults is well known, intracardiac thrombosis in children is uncommon. We report the case of a large left ventricular thrombus in an infant with ischemic cardiomyopathy secondary to anomalous origin of the left coronary artery from the pulmonary artery. Given its mobility and protrusion across the aortic valve, the patient underwent urgent thrombus removal through a transaortic approach. There were no embolic or neurologic complications. This case highlights that thrombectomy may be performed safely and successfully in critically ill pediatric patients.