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Neonates with hypoplastic left heart syndrome (HLHS) were identified from the National Inpatient Sample dataset for the years 1998-2014. These patients were stratified into two chronological groups, past group (1998-2005) and recent group (2006-2014). A total of 20,649 neonates with HLHS were identified. Of them, 9179 (44.5%) were born in the past group and 11,470 (55.5%) in the recent group. Median birth weight was significantly less in the recent group (2967 g vs. 3110 g, p = 0.005). The patients in the recent group had more patients with low birth weight (2.5 kg) and prematurity (8.7% vs 7.6% and 12.7% vs. 4.3%., respectively). In addition, recent group had more comorbidities including chromosomal anomalies, total anomalous pulmonary venous return, and kidney anomalies (5.6% vs. 3.6%, 2.3% vs. 1.7%, and 5.6% vs. 3.6%, respectively, p0.001); these were associated with a higher rate of extracorporeal membrane oxygenation utilization (9.2% vs. 4.5%, p0.001). Consequently, median length of stay was longer in the recent group (8 vs. 6 days, p0.001).Conclusion: Despite the higher frequency of comorbidities in recent group, the mortality rates decreased by 20% (from 25.3% to 20.6%, p0.001). Balloon atrial septostomy was performed less frequently in the recent group (23.3% vs. 16.1%, p0.001).What is known:• Hypoplastic left heart syndrome has the highest mortality among congenital cardiac defects during the first year of life.• Limited studies on patients' comorbidities and mortality rates trends over last two decades.What is new:• The study utilized a national database to compare in-hospital mortality and length of stay between the two time periods 1998-2005 and 2006-2014.• The recent group had more comorbidities (prematurity, chromosomal anomalies, total anomalous pulmonary venous return, and kidney anomalies), and there was higher rate of ECMO and longer length of stay, while mortality rates decreased by 20%.

Newborns with obstructed infradiaphragmatic total anomalous pulmonary venous return (TAPVR) are often clinically unstable. Temporary relief of the pulmonary venous obstruction may improve their clinical status before surgical repair. Three neonates (two premature and one full-term) with obstructed infradiaphragmatic TAPVR at the ductus venosus (DV) underwent stenting of the DV by way of an umbilical venous approach. Their clinical condition stabilized, thus allowing subsequent elective surgical repair.

Less than 7% patients with TAPVR survive into adulthood. Predictors of survive in untreated patients are: absence of concomitant CHD & atrial isomerism; large ASD; low pulmonary vascular resistance; mild/moderate TV incompetence. Experience of TAPVR repair in adults restricted by prevalence of case reports. Majority of repaired were ≥50 years old. There have been four case reports of untreated TAPVC diagnosed after 60 years of age, and three of them underwent successful surgery on 61[J Melki,1992], 63 [MH McMullan,1992] and 66 years of age[U Wetzel,2010]. Large surgical series presented by X Jian, 2012 [12 pts], S Talwar, 2008[27 pts], B Korbmacher, 2001[12 pts] and J Rodriguez-Collado, 1992 [19 pts]. Among case reports of TAPVR in adults, up to our knowledge, there are only 4 patients with TAPVR and with PA valve stenosis /RVOT obstruction.

Transesophageal echocardiography (TEE) during cardiac surgery is a routine procedure. The use of pediatric TEE probes is limited in small infants weighing less than 5 kg. Recent reports have shown the safety of monoplane intravascular ultrasound catheters in transesophageal echocardiograms. This report describes the case of a newborn with total anomalous pulmonary venous return who underwent cardiac surgery. A pre- and postbypass TEE examination was performed, with successful visualization of the cardiac anatomy and function and no complications.

A newborn with right isomerism, infracardiac total anomalous pulmonary venous return (TAPVR), and obstruction of the ductus venosus underwent stent implantation in the ductus venosus at 4 h after birth. This procedure averted early pulmonary venous obstruction. Since gradual neointimal proliferation subsequently occurred within the stent, the patient underwent stent balloon dilatation at 35 days of age and underwent surgical repair of the TAPVR with a Blalock-Taussig shunt operation at 53 days of age. Finally, the patient was discharged without any complications. Transthoracic echocardiography is very useful to confirm subsequent minimal change within the stent.

Pulmonary venous obstruction (PVO), or stenosis, is still a major complication of postoperative total anomalous pulmonary venous return, being very complex in its pathogenesis. For some types of PVO, surgery is effective, but a reoperation under cardiopulmonary bypass is generally difficult and carries a high risk. The operative balloon dilatation method performed under the guidance of transesophageal echography and fluoroscopic monitering is simple as well as safe. We describe herein the technique involved in performing this procedure through the case of a 10-month-old boy.

This paper discusses a new oblique posterior to anterior extrinsic vascular impression on the distal esophagus in total anomalous pulmonary venous return (TAPVR) below the diaphragm caused by a right common pulmonary vein.

Depiction of an echo-free space (EPS) behind the LA by M-mode and 2D echo is suggestive of total anomalous pulmonary venous return (TAPVR). 2D often shows that pulmonary veins do not drain into the LA but the pulmonary venous connections are difficult to image in respiratory distress. 2D may identify the drainage site of the common venous channel (CV). However, in TAPVR into coronary sinus (CS), dilated CS may be confused with LA. Pulsed Doppler (PD) improves the accuracy of the echo evaluation. We studied 10 patients with TAPVR with M-mode, 2D, and PD echo, before angiography. Ages ranged 1-135 days (median 19.2); 8/10 less than 7 days old. M-mode demonstrated an EPS behind LA in 6/10; LA size was small in 7/10; LV size was small in 10/10 and RV size was enlarged in 7/10; 4 had paradoxical septal motion. 2D showed EPS behind LA in 7/10 and the drainage site of the CV was identified in 7/10. PD detected continuous turbulent flow in the CV and/or at its entry site in 9/10. All were confirmed at surgery or autopsy. In one pt with TAPVR to the CS, 2D showed large RV, a small LA and LV but not EPS; PD didn't demonstrate unusual or continuous flow disturbance. Conclusion: M-mode and 2D identify the EPS representing the CV in a majority of pts with TAPVR and 2D may demonstrate drainage site of the CV. M-mode reveals a small LV in all and a small LA and large RV in a majority. Addition of PD to M-mode and 2D enhanced accuracy of detection of TAPVR and the site of drainage.