Outcomes following surgical repair of absent pulmonary valve syndrome: 30 years of experience from a Swedish tertiary referral centre.

OBJECTIVES: Absent pulmonary valve syndrome is a rare congenital heart defect with pulmonary artery dilatation and secondary airway compression. Although preoperative respiratory support and early surgical repair with pulmonary arterioplasty are often required in patients with airway compromise, the need for extensive plasty in these patients and for plasty in general in those with no or mild respiratory issues remains debatable. METHODS: We performed a retrospective survey of patients with this diagnosis and repair from 1988 to 2018. RESULTS: Twenty patients were identified. The median age and weight at repair were 0.8 (0.1-2.4) years and 7.0 (2.5-13.8) kg and included a valved conduit in 17 (85%) patients and a transannular patch in 3 patients. Five (29%) patients were ventilator-dependent prior to repair at the age of 0.3 (0.1-0.4) years. Pulmonary arterioplasty was performed in 7 patients (35%), including all 5 with ventilator dependency and 2 with respiratory symptoms due to recurrent infections. Two patients (10%) with preoperative ventilator dependency underwent extensive intrahilar arterioplasty. Preoperative ventilator dependency was associated with earlier repair and reinterventions (P < 0.05). There were 3 late deaths among cases with repair after 2000 (n = 14), none with preoperative ventilator dependency. CONCLUSIONS: The long-term outcomes of patients with this rare defect are good, comparable to those of other previous studies. Reduction pulmonary arterioplasty, which in this study was used only in patients with respiratory distress and ventilator dependency, is associated with excellent survival. Reinterventions are common in these patients.

[High prenatal detection rates of complex congenital heart defects (CHD)]. / Fler medfödda hjärtfel upptäcks med utökad screening i tre steg.

AIM: To analyze prenatal detection rates of complex CHD after the implementation of an expanded three-tiered screening model at the Skane University Hospitals in Lund and Malmö in 2015.  Methods: Retrospective review of pregnancies screened from January 1, 2015 and being born by June 30, 2018. Complex CHD was defined as needing intervention in the first year of life.  Results: In 27675 screened pregnancies, 51 out of 65 (78 %) cases of complex CHD were detected prenatally. Exclusion of isolated ventricular septal defects yielded detection rates of 93 %. All patients needing surgery within 30 days, potential univentricular hearts and D-transposition of the great arteries were identified, whilst detection rates for tetralogy of Fallot, atrioventricular septal defect and coarctation were about 90 %. CONCLUSION: Our three-tiered model results in high detection rates of complex CHD with optimized resource utilization.