Novel technique for transcatheter closure of sinus venosus atrial septal defect: The temporary suture-holding technique.

BACKGROUND: Transcatheter repair of sinus venosus atrial septal defect (SVASD) has become an alternative option to surgical repair. There are potential significant complications related to stent stability in the superior vena cava (SVC) and potential migration of the stent that need to be addressed. Therefore, the technique is still evolving. OBJECTIVES: To report results of a new modification "the suture technique" that improves safety profile of positioning and securing a covered stent in the SVC. METHODS: This is a descriptive, single center, retrospective review of patients who underwent SVASD closure using the suture technique at our institution between 02/2020 and 08/2022. RESULTS: Fourteen patients underwent transcatheter repair of SVASD using the suture technique. All procedures were successful. The suture technique allowed precise stent placement in all patients without any migration or complication. Six patients required additional stent placement at the level of the SVC. One patient had an additional covered stent placed to eliminate a tiny residual shunt. Two patients had negligible residual shunts at the time of the procedure. At follow-up, all patients clinically improved and had significant reduction in right heart size on echocardiography and/or magnetic resonance imaging. No arrhythmia was reported in any patient. None required re-intervention after a mean follow-up of 16.5 ± SD 10.5 months. CONCLUSIONS: The suture technique appears to be safe modification. Although our study involves small sample size with no comparative group, we believe our technique offers greater control over stent positioning, reducing the risk of stent embolization and residual shunting in transcatheter closure of SVASD.

Topsy-Turvy Heart with Aortopulmonary Window and Severe Airway Malacia: Prenatal Diagnosis and Review of the Literature.

The topsy-turvy heart is a very rare cardiac malformation that involves a global 90° clockwise rotation of the heart along its long axis. This rotation results in the displacement of the great arteries and severe elongation and stretching of the brachiocephalic arteries and the bronchi. We present an unusual case of topsy-turvy heart diagnosed prenatally with a large aorto-pulmonary window and. This case gives an insight into the morphological details and clinical presentation of this rare malformation and its associated complications. We also present a review of the literature of this rare anomaly showing only 15 live cases that have been published with only three cases diagnosed prenatally.

The re-occurrence of dilated cardiomyopathy in propionic acidemia after liver transplantation requiring heart transplant, first case from Middle East.

Propionic acidemia is a rare autosomal recessive inborn error of metabolism. It is relatively common in Middle East. Dilated cardiomyopathy is one of the leading causes of morbidity and mortality for patients with propionic acidemia. Liver transplantation has been used for patient with frequent metabolic decompensations and was shown to be beneficial in propionic acidemia-related dilated cardiomyopathy. Up to our knowledge, there has been one reported case of recurrent dilated cardiomyopathy 3 years after liver transplantation. We report the first case, from Middle East, of recurrent dilated cardiomyopathy, 6 years after liver transplantation.

COVID-19 related multisystem inflammatory syndrome in children (MIS-C): a case series from a tertiary care pediatric hospital in Qatar.

BACKGROUND: Multisystem Inflammatory Syndrome in Children (MIS-C) is a severe complication of coronavirus disease 2019 (COVID-19) in children, which is increasingly being reported worldwide. Here we report the first case series of 7 children diagnosed with MIS-C in Qatar. METHODS: Clinical features and outcomes of COVID-19 positive patients admitted to Sidra Medicine, Qatar from June to October 2020, who met the WHO case definition for MIS-C were reviewed. RESULTS: The mean age in our case series was 5.6 years, of which 71.4% were males. All patients were previously healthy but had a history of COVID-19 infection. Fever, rash, vomiting and abdominal pain were the most common symptoms (70-100%). The average hospitalization was 12.9 days with no case fatalities. Laboratory findings included lymphopenia and thrombocytopenia in most patients, as well as evidence of coagulopathy and elevated inflammatory markers such as C-reactive protein, ferritin and procalcitonin. Many patients (71.4%) required inotropic support in intensive care, while only one required respiratory support. Although all patients had elevated cardiac biomarkers, cardiovascular involvement was observed in 42.9% of patients with one patient developing a giant coronary aneurysm. All patients received intravenous immunoglobulin (IVIG) and 86% of patients received corticosteroids, with two patients requiring treatment with IL-1 inhibitors. CONCLUSIONS: Our report is one of the first reports on MIS-C from Asia. Although clinical features and outcomes are not significantly different from those reported elsewhere, lack of case fatalities in our cohort may indicate that early recognition and prompt medical attention is necessary for a favorable outcome in MIS-C.