A Rare Entity: Double Outlet of Both Ventricles.

Double outlet of both ventricles is an anomaly wherein both ventricles equally share the arterial trunks. A majority of the literature describes a variant of this disease with a muscular outlet septum which is perpendicular to the plane of the ventricular septum although a variant with a fibrous muscular septum can also be present. The condition may be associated with obstruction of either outflow tract, which may complicate repair. We report a child with double outlet of both ventricles and a fibrous outlet septum with unobstructed outflows. The child underwent a successful surgical repair using a two-patch technique.

Technique of Coronary Button Transfer Has no Impact on Neoaortic Root Size in Simple Transposition.

We studied the effect of various coronary transfer techniques (CTT) on neo-aortic root size after an arterial switch operation (ASO) in simple transposition by excluding the impact of recognized predisposing factors. One hundred and seventy-eight patients with simple transposition were reviewed retrospectively (January 2004-December 2018) and grouped as Punch Hole (n = 83/178), Nonpunch Hole (n = 65/178; Trapdoor or Standard) and Mixed (n = 30/178). Factors predicting the neo-aortic root z-scores- annulus, mid-sinus, and sinotubular junction (STJ) were analyzed by uni/multivariable linear regression. Follow-up was 6 years, Interquartile range (IQR) 3.4,10.6. Preoperative aortic (7.4 mm, IQR 6.9,8) and pulmonary annulus (7.5 mm, IQR 6.8,8.1) sizes were identical (P = 0.831). The changes in preoperative, postoperative, and latest median z-scores for neo-aortic annulus (-0.2, IQR -1.2,0.9; 0.0, IQR -0.9,0.9; 0.9, IQR -0.4,2.6; P < 0.001), mid-sinus (1.1, IQR-0.1,2; 2.6, IQR 1.6,3.7; 2.9, IQR 1.8,4.3; P < 0.001) and STJ (-0.1, IQR -0.8,1.1; 2.1, IQR 0.7,3; 2.4, IQR 1,3.5; P < 0.001) were significant. On multivariate analysis, preoperative pulmonary annulus z-score predicted the latest neo-aortic annulus z-score [Beta estimate (BE) = 0.32, 95% confidence interval (CI) = 0.03,0.62; P = 0.03] and STJ z-score (BE= 0.45, 95% CI= 0.20,0.70; P < 0.0001). CTT did not predict any of the latest neo-aortic z-scores (all P > 0.05). Mild plus neo-aortic regurgitation (neoAR) was not significantly different across CTT groups [punch hole 20% (n = 15/74), mixed 37% (n = 11/30), nonpunch hole 21% (n = 13/62); Fisher-exact P = 0.186], one patient required valve replacement for severe neoAR. The neo-aortic root enlarges significantly over time at all 3 levels following an ASO in simple transposition, however, this is not significantly influenced by the CTT utilized.

Prenatal diagnosis and planned peri-partum care as a strategy to improve pre-operative status in neonates with critical CHDs in low-resource settings: a prospective study.

BACKGROUND: Prenatal diagnosis and planned peri-partum care is an unexplored concept for care of neonates with critical CHDs in low-middle-income countries. OBJECTIVE: To report the impact of prenatal diagnosis on pre-operative status in neonates with critical CHD. METHODS: Prospective observational study (January 2017-June 2018) in tertiary paediatric cardiac facility in Kerala, India. Neonates (<28 days) with critical CHDs needing cardiac interventions were included. Pre-term infants (<35 weeks) and those without intention to treat were excluded. Patients were grouped into those with prenatal diagnosis and diagnosis after birth. Main outcome measure was pre-operative clinical status. RESULTS: Total 119 neonates included; 39 (32.8%) had prenatal diagnosis. Eighty infants (67%) underwent surgery while 32 (27%) needed catheter-based interventions. Pre-operative status was significantly better in prenatal group; California modification of transport risk index of physiological stability (Ca-TRIPS) score: median 6 (0-42) versus 8 (0-64); p < 0.001; pre-operative assessment of cardiac and haemodynamic status (PRACHS) score: median 1 (0-4) versus 3 (0-10), p < 0.001. Age at cardiac procedure was earlier in prenatal group (median 5 (1-26) versus 7 (1-43) days; p = 0.02). Mortality occurred in 12 patients (10%), with 3 post-operative deaths (2.5%). Pre-operative mortality was higher in postnatal group (10% versus 2.6%; p = 0.2) of which seven (6%) died due to suboptimal pre-operative status precluding surgery. CONCLUSION: Prenatal diagnosis and planned peri-partum care had a significant impact on the pre-operative status in neonates with critical CHD in a low-resource setting.