Aorto-Left Ventricular Tunnel - Prenatal Diagnosis and Outcome.

PURPOSE: Aorto-left ventricular tunnel (ALVT) is an extremely rare, albeit prenatally detectable, extracardiac channel that connects the ascending aorta to the cavity of the left ventricle. MATERIALS AND METHODS: All ALVTs diagnosed prenatally (2006-2020) in five tertiary referral centers were retrospectively assessed for prenatal ultrasound findings, intrauterine course, postnatal outcome, and surgical treatment. We focused on the size of the tunnel and alterations of perfusion of the left ventricular outflow tract and aortic arch. RESULTS: 11 fetuses were diagnosed with ALVT at a mean gestational age of 24.8 weeks. All cases were associated with severe dilatation of the left ventricle and a to-and-fro flow in the left outflow tract. Signs of congestive heart failure were present in five fetuses, four of which were terminated and one of which died in the neonatal period. One fetus died in utero at 34 weeks without prior signs of cardiac failure. Of the five survivors, two underwent the Ross procedure. In both cases the prenatal left ventricular outflow was exclusively via a large tunnel. The remaining three neonates underwent patch closure of the tunnel. In these cases, the prenatal outflow of the left ventricle was via the aortic valve and simultaneously over the tunnel. CONCLUSION: Prenatal diagnosis of ALVT should be considered in the presence of left ventricular hypertrophy, dilatation of the aortic root, and to-and-fro flow in the aortic outflow tract. Signs of heart failure are associated with an unfavorable outcome. Large tunnels, particularly in combination with the absence of flow over the aortic valve, may be an unfavorable predictor of surgical repair.

Fetal Echocardiography in Predicting Postnatal Outcome in Borderline Left Ventricle.

OBJECTIVES: Prenatal prediction of postnatal univentricular versus biventricular circulation in patients with borderline left ventricle (bLV) remains challenging. This study investigated prenatal fetal echocardiographic parameters and postnatal outcome of patients with a prenatally diagnosed bLV. METHODS: We report a retrospective study of bLV patients at four prenatal centers with a follow-up of one year. BLV was defined as z-scores of the left ventricle (LV) between -2 and -4. Single-ventricle palliation (SVP), biventricular repair (BVR), and no surgical or catheter-based intervention served as the dependent outcome. Prenatal ultrasound parameters were used as independent variables. Cut-off values from receiver operating characteristic curves (ROC) were determined for significant discrimination between outcomes. RESULTS: A total of 54 patients were diagnosed with bLV from 2010 to 2018. All were live births. Out of the entire cohort, 8 (15 %) received SVP, 34 (63 %) BVR, and 12 (22 %) no intervention. There was no significant difference with regard to genetic or extracardiac anomalies. There were significantly more patients with endocardial fibroelastosis (EFE) in the SVP group compared to the BVR group (80 % vs. 10 %), (p < 0.001). Apex-forming LV (100 % vs. 70 %) and lack of retrograde arch flow (20 % vs. 80 %) were associated with no intervention (p < 0.001). With respect to BVR vs. SVP, the LV sphericity index provided the highest specificity (91.7 %) using a cutoff value of ≤ 0.5. CONCLUSION: The majority of bLV patients maintained biventricular circulation. EFE, retrograde arch flow, and LV sphericity can be helpful parameters for counseling parents and further prospective studies can be developed.

Prenatal Diagnosis and Postnatal Outcome of Eight Cases with Criss-Cross Heart - A Multicenter Case Series.

OBJECTIVE: Criss-cross heart (CCH) is a rare congenital cardiac defect defined by crossing of ventricular inflow streams contributing to less than 0.1 % of all congenital heart anomalies. Due to its rarity and complexity, prenatal diagnosis in these patients remains challenging. We sought to describe prenatal findings and postnatal course in eight cases of prenatally diagnosed CCH. METHODS: This is a retrospective case series of prenatally diagnosed CCH in three centers between 2010-2017. We reviewed fetal echocardiograms as well as postnatal clinical charts and surgical reports. RESULTS: 8 cases of CCH were included. The median gestational age at diagnosis was 27 weeks. 7 patients were found with situs solitus, one fetus with situs ambiguous. In all patients, the four-chamber view was abnormal. There was atrioventricular discordance in half of the patients, while all patients showed ventriculoarterial discordance. All patients were found with additional cardiac anomalies, including ventricular septal defect, double outlet right ventricle, right aortic arch, atrial septal defect and pulmonary stenosis. Three patients underwent amniocentesis without pathological findings. All patients were born alive at a median gestational age of 38 + 2 weeks and survived our median follow-up of 181 days. CONCLUSION: CCH can be diagnosed prenatally by detailed fetal echocardiography when observing an abnormal four-chamber view with crossing of inflow streams into both ventricles and a lack of parallel orientation of the atrioventricular valve axis due to a clockwise or counterclockwise rotation of the ventricular mass along its axis. With the help of prenatal ultrasound, parental guidance and counselling as well as postnatal pediatric cardiac management can be warranted.

Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome.

OBJECTIVES: To analyse prenatal parameters predicting biventricular (BV) outcome in pulmonary atresia with intact ventricular septum/critical pulmonary stenosis (PAIVS/CPS). METHODS: We evaluated 82 foetuses from 01/08 to 10/18 in 3 centres in intervals 1 (< 24 weeks), 2 (24-30 weeks) and 3 (> 30 weeks). RESULTS: 61/82 (74.4%) were livebirths, 5 (8.2%) lost for follow-up, 3 (4.9%) had compassionate care leaving 53 (64.6% of the whole cohort and 86.9% of livebirths) with intention to treat. 9 died, 44/53 (83.0%) survived. 24/38 (63.2%) with information on postnatal outcome had BV outcome, 14 (36.8%) non-BV outcome (2 × 1.5 circulation). One with BV outcome had prenatal valvuloplasty. Best single parameter for BV outcome was tricuspid/mitral valve (TV/MV) ratio (AUC 0.93) in intervals 2 and 3 (AUC 0.92). Ventriculo-coronary-arterial communications (VCAC) were present in 11 (78.6%) in non-BV outcome group vs. 2 (8.3%) in BV outcome group (p < 0.001). Tricuspid insufficiency (TI)-Vmax > 2.5 m/s was present in BV outcome group in75.0% (18/24) vs. 14.3% (2/14) in non-BV outcome group. Including the most predictive markers (VCAC presence, TI- Vmax < 2.5 m/s, TV/MV ratio < cutoff) to a score, non-BV outcome was correctly predicted when > 1 criterion was fulfilled in all cases. After recently published criteria for foetal intervention, only 4/9 (44.4%) and 5/14 (35.7%) in our interval 2 + 3 with predicted non-BV outcome would have been candidates for intervention. Two (1 × intrauterine intervention) in interval 2, two in interval 3 reached BV outcome and one 1.5 circulation without intervention. CONCLUSION: TV/MV ratio as simple parameter has high predictive value. After our score, non-BV outcome was correctly predicted in all cases. Criteria for foetal intervention must further be evaluated.

Prenatal Diagnosis and Outcome of Congenital Corrected Transposition of the Great Arteries - A Multicenter Report of 69 Cases.

OBJECTIVE: Congenital corrected transposition of the great arteries (ccTGA) is a rare congenital cardiac anomaly which remains difficult to diagnose prenatally. We aim to investigate the natural history, associated anomalies and the outcome of patients in prenatally diagnosed ccTGA. METHOD: This was an international multicenter retrospective analysis of fetuses with a diagnosis of ccTGA from 2002 to 2017. We reviewed clinical and echocardiographic databases of seven centers. Anatomic survey and fetal echocardiography were performed according to international guidelines of ISUOG. RESULTS: We considered 69 fetuses with prenatally suspected ccTGA. There was an overall survival rate of 91 % among 54 patients with a confirmed diagnosis. Survival to live birth was 96 % (52/54) and survival on an intention-to-treat basis was 94 % (49/52). The mean gestational age at the time of diagnosis was 25.6 ±â€Š5.9 weeks of gestation. In 7 out of 54 fetuses (13 %), ccTGA was an isolated finding. Dextro/mesocardia was present in 15 cases (27.8 %). Intracardiac anomalies were present in 46/54 cases (85.2 %) with the most frequent anomaly being a ventricular septal defect present in 41 fetuses (75.9 %). Complete heart block was diagnosed in 10 cases (18.5 %). Extracardiac anomalies were observed in 9 out of 54 cases (16.7 %). Prenatal karyotyping of the fetus was available in 30/54 (55.6 %) cases with chromosomal anomalies in 4/30 (13.3 %). CONCLUSION: ccTGA is a rare cardiac anomaly often accompanied by a variable spectrum of further intracardiac abnormalities. Accurate diagnosis of ccTGA, which can be integrated into parental counselling, is feasible with a favorable short-term outcome for affected neonates.

A Successful Repair of Pseudocoarctation With Associated Aortic Arch Aneurysm in an Eight-Month-Old Infant.

Pseudocoarctation of the aorta is an uncommon congenital entity characterized by juxtaductal elongation and tortuosity of the aortic arch. It is known to be associated with aneurysm formation. Our patient is a 7.7-month-female infant followed prenatally by magnetic resonance imaging that detected this anomaly. Subsequently, postnatal preoperative computed tomography angiography was confirmatory. The operation comprised resection of the aneurysmal portion of the aorta and extended end-to-end reconstruction, without prosthesis usage. The left subclavian artery was reimplanted into the left carotid artery. The postoperative course was uneventful. Computed tomography aortography and transthoracic echocardiography demonstrated no residual stenosis at six months of follow-up.

Anomalous Origin of the Circumflex Coronary Artery From the Right Pulmonary Artery Associated With Anomalous Aortic Origin of the Right Coronary Artery: A Case Report of Surgical Treatment.

We present a patient with the extremely rare coexistence of two distinct coronary artery malformations: anomalous aortic origin of the right coronary artery from the left aortic sinus with intramural course and anomalous origin of the circumflex coronary artery from the right pulmonary artery. Surgical reimplantation of the anomalous left circumflex coronary artery to the aorta and unroofing of the intramural portion of the anomalous right coronary artery were performed.

Single-centre 20-year experience with repair of truncus arteriosus.

OBJECTIVES: We analysed a large series of truncus arteriosus repairs with a focus on early and late outcomes. METHODS: Ninety-seven consecutive patients who underwent truncus arteriosus repair (1997-2017) were included retrospectively. Univariable analysis for mortality and reintervention was performed. RESULTS: The early mortality rate decreased from 45% (1997-2007; 14/31) to 4.5% (2008-2017; 3/66) (P = 0.001). Repair beyond the neonatal period (P = 0.03) and direct connection for right ventricular outflow tract reconstruction (P = 0.001) were associated with early death by univariable analysis. Overall survival was 68 ± 6.0% at 15 years; a majority of the deaths (90%; 9/10) occurred within the first year after repair. Freedom from the first and second conduit reoperations at 10 years was 22.9% and 89%, respectively. Freedom from truncal valve (TrV) reoperation was 83.9% at 15 years. Initial TrV insufficiency ≥ moderate was associated with a TrV reoperation (P = 0.008) with freedom from TrV reoperation in this subgroup of 58.3% at 10 years. Freedom from TrV reoperation for quadricuspid and tricuspid TrVs was 66.8% and 93.8% at 10 years with 100% for bicuspid TrVs at 8 years. At the last follow-up, 98.5% (69/70) were in New York Heart Association functional class I-II. CONCLUSIONS: In the current era, truncus arteriosus can be repaired with a low early mortality rate and a good long-term outcome. A significant reintervention burden still persists. Direct connection is associated with early mortality.

Absent pulmonary valve syndrome - diagnosis, associations, and outcome in 71 prenatally diagnosed cases.

OBJECTIVE: The objective of this study was to analyze the spectrum of prenatally diagnosed absent pulmonary valve syndrome (APVS) and the outcome from diagnosis onwards. Fetuses with APVS and tetralogy of Fallot (TOF/APVS) and with APVS and intact ventricular septum (APVS/IVS) were included. METHOD: Multicenter retrospective study of the International Prenatal Cardiology Collaboration Group. Clinical and echocardiographic databases of nine referral centers were reviewed from 2012-2016. RESULTS: The cohort included 71 cases, 59 with TOF/APVS and 12 with APVS/IVS. In 18.3% of cases, diagnosis was achieved within first trimester. Association with hydrops fetalis was high within first trimester (69%). No fetus with known outcome survived after first trimester diagnosis. Karyotype anomalies occurred in 45% of cases with known karyotype. Intrauterine fetal demise occurred in 14.3%. Overall survival after initial diagnosis in the total cohort was 28.1% (28.8% TOF/APVS and 25.0% APVS/IVS). Survival to birth was 50% in TOF/APVS and 44.4% in APVS/IVS. Survival of subjects born alive beyond neonatal period was 84.6% in TOF/APVS and 100% in APVS/IVS. CONCLUSION: Diagnosis of APVS is feasible within first trimester. Outcomes remain guarded, especially if first trimester diagnosis is included into the analysis because of associated karyotypic anomalies, the presence of hydrops fetalis, and patent ductus arteriosus. © 2017 John Wiley & Sons, Ltd.

Prenatal diagnosis of left pulmonary artery-to-pulmonary vein fistula and its successful surgical repair in a neonate.

Pulmonary arteriovenous fistula is a rare disease. To the best of our knowledge, prenatal diagnosis of a fistula between the left pulmonary artery and the left pulmonary vein has not been described in the medical literature. We report a case of the prenatal diagnosis of a left pulmonary artery-to-pulmonary vein fistula, followed by successful neonatal surgical repair.

Open cardiac surgery in the first hours of life using autologous umbilical cord blood.

OBJECTIVE: This article describes the first clinical experience of complete repair of complex critical congenital heart diseases (CHDs) in the first hours of life using autologous umbilical cord blood (UCB). Prenatal diagnosis and harvesting of autologous UCB allow to modify perioperative management and to perform corrective surgery in the first hours of a patient's life. This approach can afford avoiding homologous blood transfusion and preventing development of hypoxemia and heart failure due to hemodynamic changes of complex critical CHD. METHODS: The study group included 14 consecutive prenatally diagnosed patients with critical complex CHD during the period from September 2009 to August 2010. Autologous UCB was harvested in accordance to NetCord-FACT International Standards for Cord Blood Collection and was used during the surgery with cardiopulmonary bypass (CPB). In all cases, complete repair was performed during the first hours of life: arterial switch operation (n=9); arterial switch operation with total anomalous pulmonary venous communication repair (n=1); arterial switch operation with interruption of the aortic arch repair (n=1); Ebstein's repair (n=2); and aortopulmonary window repair with interruption of the aortic arch repair (n=1). All procedures were performed using moderate hypothermia with cold-crystalloid cardioplegia, except one case that required deep hypothermic circulatory arrest. RESULTS: A mean of 92±16 ml of UCB was harvested. Autologous UCB was used during the surgery in all 14 cases. Mean age of newborns at operation was 4.7±2 h (3-8). No patients required intensive care unit (ICU) admission, interventional procedures, mechanical ventilation, or medications before surgery. Twelve patients underwent bloodless open heart surgery; eight of them completely avoided homologous blood transfusion during the perioperative period. There was one postoperative death in our study (Ebstein's anomaly). CONCLUSIONS: The use of autologous umbilical cord blood is feasible in neonatal open heart surgery. Complete surgical repair of complex critical CHD can be applied successfully to neonates within the first hours of life.