Tachyarrhythmias originating in the atrioventricular (AV) node and AV junction including the bundle of His complex (BH) are called junctional tachycardia (JT) or junctional ectopic tachycardia (JET). Congenital JET (CJET) is a rare arrhythmia that occurs in patients without a preceding cardiac surgery and can be refractory to medical therapy and associated with high morbidity and mortality. CJET has a high rate of morbidity and mortality with death occurring in 35% of cases. JET occurring within 72 hours after cardiac surgery is referred to as postoperative JET (POJET) and caused by direct trauma, ischemic, or stretch injury to the AV conduction tissues during surgical repair of congenital heart defects. Focal junctional tachycardia (FJT) is also known as automatic junctional tachycardia and includes paroxysmal or non‐paroxysmal forms. We discuss a staged approach to therapy with improved pharmacological therapies and the use of catheter‐based therapies.

With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, post-double switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ≤0.9 g/dL) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.

Rotation of the testis around the axis of the spermatic cord results in tissue ischaemia and testicular torsion (TT). TT in the newborn infant in the 1 ^st month of life is referred to as neonatal TT (NTT) or perinatal TT and occurs in 6.1/100, 000 live births. The true incidence could be higher as some of these occur prenatally and can be asymptomatic. TT can be extravaginal, intravaginal and mesorchial and NTT is usually extravaginal. Physical examination can be adequate for the diagnosis, and utility of ultrasound (US) is mainly to exclude other conditions. If the timing of the torsion is prenatal, the testicle may not be salvageable. But, in certain situations, these could be asymptomatic bilateral TT. When the timing of torsion is not simultaneous (asynchronous torsion) early contralateral orchiopexy done at the time of exploration would prevent the occurence of asynchronous torsion. Non-operative maneuvres to detorse in NTT are not successful and not recommended. This review focuses on the diagnostic approach and management.

Caudal Regression Syndrome (CRS) or Caudal dysgenesis syndrome (CDS) is characterized by maldevelopment of the caudal half of the body with variable involvement of the gastrointestinal, genitourinary, skeletal, and nervous systems. CRS affects 1–3 newborn infants per 100,000 live births. The prevalence in infants of diabetic mothers is reported at 1 in 350 live births which includes all the variants. A related condition is sirenomelia sequence or mermaid syndrome or symmelia and is characterized by fusion of the legs and a variable combination of the other abnormalities. The Currarino triad is a related anomaly that includes anorectal atresia, coccygeal and partial sacral agenesis, and a pre-sacral lesion such as anterior meningocele, lipoma or dermoid cyst. A multidisciplinary management approach is needed that includes rehabilitative services, and patients need a staged surgical approach.