The chance of spontaneous patent ductus arteriosus closure in preterm infants born before 32 weeks of gestation is high and continues to increase until 5 years of follow-up.

AIM: The primary aim was to estimate premature infants' spontaneous patent ductus arteriosus closure rate. Secondly, to identify criteria associated with the chance of spontaneous closure. METHODS: We performed a retrospective cohort study of 167 infants born before 32 weeks of gestation and diagnosed with a patent ductus arteriosus between 1 January 2008 and 31 December 2017. The spontaneous patent ductus arteriosus closure event rate was evaluated using the Kaplan-Meier estimator. RESULTS: The spontaneous closure rate within the first year of life was 66% (95% CI 58%-73%), increasing to 80% (95% CI 72%-86%) five years after birth. When including both spontaneous closure and closure following treatment, 96% (95% CI 86%-100%) closed within 5 years after birth. The chance of spontaneous closure was reduced in the case of a large patent ductus arteriosus: OR 0.16 (95% CI 0.05-0.52), left atrial enlargement: OR 0.16 (95% CI 0.05-0.51), and pulmonary hypertension: OR 0.23 (95% CI 0.07-0.74). CONCLUSION: The chance of spontaneous closure in premature infants born between 23 and 32 weeks of gestation was high, and the incidence continued increasing until 5 years of follow-up.

[Bannayan-Riley-Ruvalcaba syndrome and juvenile polyposis in a two-year-old girl]. / Bannayan-Riley-Ruvalcabas syndrom og juvenil polypose hos en toårig pige.

Bannayan-Riley-Ruvalcaba syndrome is a rare disease, which is characterized by macrocephaly, benign hamartomas, lipomas, haemangiomas, pigmented maculae, developmental delay and mental retardation. This case report describes how the combination of macrocephaly, hypertelorism, high palate and intestinal polyposis led to the diagnosis of this syndrome in a two year and seven month-old girl. The diagnosis was confirmed by molecular genetic analysis showing deletion of the entire PTEN gene and the majority of the neighbouring gene BMPR1A, which predisposes to juvenile polyposis.

[Cystic tumour of the atrioventricular node in a nine year-old girl]. / Cystisk tumor i atrioventrikulærknuden som dødsårsag hos en niårig pige.

Cystic tumour of the atrioventricular node (CTAVN) is a rare type of cardiac tumour. We report a case of a previously healthy nine year-old girl, presenting with syncope caused by complete AV block. Despite intensive treatment, she died of multiple organ failure after two days. Infectious and congenital AV block were ruled out. Histology of the conduction system showed that a microscopic CTAVN was the cause of death. CTAVN is a rare cause of AV block and cardiac arrest, but should be considered in cases of unexplained death.

[Tricuspid valve annuloplasty]. / Plastik på trikuspidalklappen.

INTRODUCTION: Tricuspid valve regurgitation (TVR) is often secondary to left-sided or congenital heart disease (CHD). Surgical correction of TVR is indicated when the primary abnormalities require operation. MATERIAL AND METHODS: Retrospective analysis of all 50 patients (mean age: 65 years (range: 24-83), 27 males, 23 females) operated with tricuspid valve annuloplasty (TVA) in our hospital from 2000 to 2007. TVA took place concomitantly with operation for left heart disease (n = 39, 78%) or CHD (n = 11, 22%). RESULTS: Preoperatively, TVR was severe in 40, moderate in six and mild in four cases. Thirty-four had annulus dilation > 2 cm/m(2) body surface area. Half of the patients were in New York Heart Association (NYHA) class III or IV. Postoperatively, TVR was severe in two cases, moderate in one, mild in 25 and absent in 18, and 95% of patients followed up were in NYHA I or II. There were five (10%) early and two (4%) late deaths. Temporary arrhythmias requiring medical treatment occurred in 35 cases, but eight required permanent pacemaker. No tricuspid valve reoperations were performed. CONCLUSIONS: The combination of correction of primary heart abnormalities and TVA is associated with high perioperative mortality. In survivors, postoperative clinical improvement is common, in all probability this is mainly due to the correction of the primary disease. TVA reduces TVR, but its precise contribution to clinical improvement and prognosis remains unknown.

Blood flow measured by magnetic resonance imaging at rest and exercise after surgical bypass of aortic arch obstruction.

INTRODUCTION: Blood flow distribution after ascending-to-descending aortic bypass in complex aortic arch obstruction is poorly described. OBJECTIVE: To study blood flow by magnetic resonance (MR) imaging at rest and during exercise in patients with aortic arch obstruction and a bypass tube and in healthy controls. MATERIAL AND METHODS: Seven patients (median 18 years (range: 14-54 years) and weight 79 kg (range 51-91 kg)) were studied 25 months (range 6-68 months) following surgical insertion of 14- or 16-mm bypass tube from the ascending to the distal descending thoracic aorta. Seven sex- and aged-matched normotensive subjects served as controls. MR real-time flow was measured in the ascending aorta and the proximal descending thoracic aorta in all participants and in the bypass tube in patients at rest and during supine leg exercise at 0.5 and 1.0 W kg(-1). RESULTS: Ascending aortic flow at rest in patients was 2.9l min(-1)m(-2) (range 2.3-4.4) and increased with exercise to 5.3 (range 4.3-7.3) at 1.0 W kg(-1), which was not different from controls (3.4 (range 2.4-4.6) and 6.1 (range 5.0-6.9)). The bypass tube carried roughly the same flow as the proximal descending aorta at rest (1.5 (range 0.7-2.0) and 1.0 (range 0.2-2.0), respectively, and flows increased similarly during exercise (2.8 (range 1.5-4.0) and 2.0 (range 0.8-4.1), respectively at 1.0 W kg(-1)). Flow to the upper body did not differ between groups nor changed with supine leg exercise. CONCLUSION: With aortic arch obstruction, an ascending-to-descending aortic bypass tube provides normal flow to the lower body at rest and during supine leg exercise without evidence of steal from the upper body.

[Multiple pregnancies in women with corrected tetralogy of Fallot]. / Multipel graviditet hos kvinder med korrigeret Steno-Fallots tetralogi.

Tetralogy of Fallot is the most common cyanotic heart disease accounting for 5 to 8% of all cases of congenital heart disease. In a cohort of women with corrected Tetralogy of Fallot, followed concerning fertility and pregnancy outcome, we observed two cases of uncomplicated multiple pregnancies. Such pregnancies have not previously been described. Despite the overload on the operated heart resulting from a multiple pregnancy, both women experienced uncomplicated pregnancies and deliveries of healthy children. This reflects the good hemodynamic results of complete repair and meticulous pregnancy follow-up.