Primary Arterial Switch Operation for Late Presentation of Transposition of the Great Arteries With Intact Ventricular Septum.

Transposition of the great arteries (TGA) with intact ventricular septum (IVS) are subject to neonatal arterial switch operation (ASO) to avoid deconditioning of the left ventricle (LV). Often an early repair is not feasible in developing countries where late referral is frequent. We aim to explore ASO outcomes in late TGA-IVS and compare "unfavorable candidates" (LV myocardial mass <35gr/m2 unfavorable geometry. banana-like LV shape) with other late TGA-IVS. Single-center retrospective study on late TGA-IVS who underwent primary ASO between 2015 and 2018. We divide patients into 2 groups: unfavorable candidates and favorable candidates TGA. We report categorical variables as number and percentage and continuous variables as median with interquartile range. Differences were assessed with Chi-squared or Fisher exact tests, Wilcoxon sign-rank, and Wilcoxon-rank sum tests. 45 TGA-IVS were referred with a median age of 35 days. Pre-operative echocardiography identified banana-like LV shape in 66.7%; unfavorable LV geometry in 47.6% and LV mass <35gr/m2 in 51%. Only 1 death occurred related to myocardial ischemia. Five patients (11%) required ECMO for LV dysfunction, with unfavorable candidates having a higher but not significant use (18.7% vs 6.9%, P = 0.33). At discharge, echocardiography demonstrated significant LV mass improvement compared to pre-operative (58.6 vs 33.8 gr/m2, P < 0.0001) with no significant echocardiographic difference between unfavorable and favorable late-TGA. Primary ASO in late presenter TGA-IVS can be performed safely with low mortality having a low threshold to ECMO. Significantly unconditioned LV are amenable with primary ASO with good LV mass recovery.

A Misdiagnosed Case of Double Outlet Right Atrium Associated With Hypoplastic Right Ventricle.

Double outlet right atrium (DORA) is a rare congenital heart disease in which the right atrium opens into both ventricles. The reduced leftward motion of the interventricular septum causes a malalignment between the atrial and the ventricular septum at the cardiac crux, which is the pathognomonic feature of this heart defect. We describe a case of significant exertional desaturation in an adult patient who was diagnosed with DORA, restrictive right ventricle, and anomalous tricuspid valve. Subsequently, the patient underwent one-and-a-half ventricular palliation.

How to deal with atrial septal defect closure from right internal jugular vein: Role of venous-arterial circuit for sizing and over-the-wire device implantation.

Secundum atrial septum defect (ASD) is the most common congenital heart disease. It is usually treated by a transcatheter approach using a femoral venous access. In case of bilateral femoral vein occlusion, the internal jugular venous approach for ASD closure is an option, in particular in cases where ASD balloon occlusion test and sizing is needed. Here, we report on a new technique for ASD closure using a venous-arterial circuit from the right internal jugular vein to the femoral artery. Two patients (females, 4 and 10 years of age) had occlusion of both femoral veins because of a previous history of pulmonary atresia and intact ventricular septum, for which they underwent percutaneous radiofrequency perforation and balloon angioplasty. These subjects needed balloon occlusion test of a residual ASD to size the hole and to check for hemodynamic suitability to ASD closure. After performing a venous-arterial circuit, a 24 mm St Jude ASD sizing balloon catheter was advanced over the circuit and the defect closed for 15 min to check hemodynamics and size the defect. ASD was closed is hemodinamically suitable. This technique was safe and reliable. © 2016 Wiley Periodicals, Inc.

Goose-neck snare-assisted transcatheter ASD closure: A safety procedure for large and complex ASDs.

OBJECTIVE: To report on a new technique that increases the safety of percutaneous atrial septal defect (ASD) closure using a goose-neck snare system. BACKGROUND: ASD transcatheter closure is a widespread procedure. However, in some cases, ASDs may be large and with soft rims. In these situation, a potential risk exists for device malposition or embolization. METHODS: When transesophageal echocardiography (TEE) evaluation and balloon sizing showed large defects with floppy rims the chosen Amplatzer device was implanted in a standard way. In large defects with floppy rims, before release a 5-mm goose-neck snare with its 4 Fr catheter was placed across the delivery cable and fixed to catch the screwing mechanism of implanted Amplatzer device. The delivery cable was unscrewed and the device reached its final position without any tension. If the position was considered satisfactory the device was released from the goose-neck snare. RESULTS: Thirteen patients had a snare-assisted ASD transcatheter closure. Median device size was 24 mm (range 14-38 mm). Retrieval or repositioning of the device using the goose-neck snare was performed in four cases: in three patients, because of device malposition after delivery cable release and in one patient, because of unsuitability of closure of a second significant defect. Furthermore, in two subjects with multiple ASDs, a second fenestration looked quite significant with the device still attached to the delivery cable while it appeared smaller after release. CONCLUSIONS: Snare-assisted Amplatzer ASD device placement is a new method for ASD percutaneous closure and adds safety to the procedure.

Patent ductus arteriosus balloon sizing: A new technique to evaluate the size in complex cases.

Patent ductus arteriosus (PDA) transcatheter closure is a widespread procedure. However in some cases PDA measurements may be unclear and choice of the proper device could be quite difficult. This may happen in large PDA and in particular in adults. We have developed a new technique using an ASD sizing balloon to measure the PDA in order to better understand PDA anatomy and size. The first step is to create an artero-venous circuit across the PDA. A 24 or a 34 mm Amplatzer balloon sizing for ASD closure is placed over the wire from the venous access in the descending aorta. Then, the balloon is inflated and gently pulled back across the PDA toward the pulmonary artery. The frame where the balloon is exactly across the PDA is chosen and measurements performed. In conclusion, a new method for PDA measurement in large PDA is reported. The procedure is safe and reliable. © 2015 Wiley Periodicals, Inc.

The Edwards Valeo lifestents in the treatment and palliation of congenital heart disease in infants and small children.

BACKGROUND: Intravascular stenting is the procedure of choice in the treatment of vascular stenoses. However, in infants and small children large sheaths are needed, and adult-size stents cannot be implanted. The Valeo Biliary Lifestent (Edwards Lifesciences Irvine, CA) is low profile and can be dilated up to 18-20 mm. We aimed to report on early and mid-term results with the use of Valeo stents in infants and children with congenital heart disease. METHODS: Twenty-five subjects were treated in our experience. Mean age and weight were 39 ± 35 months (range 1-132) and 10.4 ± 6.7 kg (range 3-30), respectively. Two groups of patients were: Group A: patients with pulmonary artery stenosis (21 subjects); Group B subjects in whom stenting was applied in other vessel or heart sites (four subjects). RESULTS: A total of 35 stents were successfully used. Fluoroscopy time was 32 ± 11 min. No intra-operative death or hospital mortality was recorded. Stent post dilatation was performed in nine patients. The incidence of complication was 12% (3/25) (two subjects developed transient hypotension and bradycardia which required inotropic treatment, 1 patient developed mild lung bleeding). There was a significant improvement of angiographic appearance and RV pressure (RV/AO systolic pressure ratio before 1 ± 02 (range 0.8-1.5) versus after the procedure 0.6 ± 0.2 (range 0.4-0.9) P < 0.001). In group B stents were successfully implanted in aortic recoarctation, interatrial septum and in two modified BT shunts. At a median follow-up of 18 months (range 1-24 months) results remained stable and no complications occurred. In particular no stent fractures were seen. Furthermore, redilation was performed safely and successfully in three subjecs up to 18 months after the first implantation. CONCLUSIONS: In our series, Valeo Lifestents have proven to be effective and with low incidence of complication in various anatomical settings, in low weight infants and in early post-operative course. Large series and longer follow-up are mandatory.

[Anomalous origin of the coronary arteries: five case reports and review of the literature]. / Origine anomala delle coronarie: cinque casi clinici e revisione della letteratura.

BACKGROUND: Although individual patient outcomes are highly variable, coronary artery anomalies may be associated with sudden cardiac death or acute coronary syndrome. METHODS: We report 5 cases of anomalous origin of coronary arteries: a single coronary artery originating from the right sinus of Valsalva, a case of isolated anomalous right coronary artery originating separately from the left sinus of Valsalva, a case of right coronary artery originating between the right and left sinus of Valsalva, a case of anomalous left circumflex artery originating from the right sinus of Valsalva, and a case of anomalous left anterior descending coronary artery originating from the right sinus of Valsalva. RESULTS: Although in one case the right coronary artery runned between the aorta and the right pulmonary artery, no significant alterations due to coronary artery compression or atheromatous lesions at the anomalous coronary ostia were observed. In all reported cases, symptoms were different as a result of cardiovascular comorbidities. All coronary anomalies were identified at coronary angiography, which was performed for other indications. CONCLUSIONS: The identification of a clear correlation between symptoms and coronary artery anomalies seems challenging in clinical practice. However, it is crucial to confirm or rule out the presence of coronary compression caused by the anomalous origin of coronary arteries.