Hybrid Palliation for Hypoplastic Borderline Left Ventricle: One More Chance to Biventricular Repair.

Treatment options for hypoplastic borderline left ventricle (LV) are critically dependent on the development of the LV itself and include different types of univentricular palliation or biventricular repair performed at birth. Since hybrid palliation allows deferring major surgery to 4-6 months, in borderline cases, the decision can be postponed until the LV has expressed its growth potential. We aimed to evaluate anatomic modifications of borderline LV after hybrid palliation. We retrospectively reviewed data from 45 consecutive patients with hypoplastic LV who underwent hybrid palliation at birth between 2011 and 2015. Sixteen patients (mean weight 3.15 Kg) exhibited borderline LV and were considered for potential LV growth. After 5 months, five patients underwent univentricular palliation (Group 1), eight biventricular repairs (Group 2) and three died before surgery. Echocardiograms of Groups 1 and 2 were reviewed, comparing LV structures at birth and after 5 months. Although, at birth, all LV measurements were far below the normal limits, after 5 months, LV mass in Group 2 was almost normal, while in Group 1, no growth was evident. However, aortic root diameter and long axis ratio were significantly higher in Group 2 already at birth. Hybrid palliation can be positively considered as a "bridge-to-decision" for borderline LV. Echocardiography plays a key role in monitoring the growth of borderline LV.

[Proposal of a common model for informed consent for interventional procedures in congenital heart disease patients]. / Proposta di un modello comune al consenso informato per le procedure di emodinamica nel cardiopatico congenito.

BACKGROUND: A multidisciplinary study group involving physicians and jurists was established to review and approve an informed consent about the most frequent interventional procedures for congenital heart diseases. METHODS: The authors worked together with representatives of the Italian Society of Pediatric Cardiology and Congenital Heart Disease (SICP) Council and Jurist's expert in the field of health case-law. The final draft was shared with the major Italian centers involved in congenital interventional procedures and with AICCA, the Italian Patients Association of Congenital Heart Diseases - Adults and Children. RESULTS: At the end of this review process, a final informed consent form was developed for the most frequent procedures performed in our catheterization laboratories. All of them consist of two parts: a general statement and a procedure-related one. CONCLUSIONS: The work performed by this multidisciplinary study group, under the supervision of the SICP, resulted in a new dedicated informed consent about interventional procedures in the field of congenital cardiology, taking into account the new legal requests. This informed consent is intended to be both a document that can be used as such and a document from which to derive a specific document for each center. We believe that using similar informed consents in all Congenital Heart Disease Centers or at least have informed consents all inspired by the same setting, could be a further improvement in taking care of the patients and their families.

Case Report: "Smart Palliation" and "Clepsydra Shape": A new approach in complex congenital heart disease.

A limiting factor in using vascular conduits in the pediatric/newborn population is their inability to grow. Many complex congenital heart diseases require palliative surgery, but using rigid and nonexpandable conduits does not allow the structures to grow and anticipates the need for redo surgery. In newborns, a way to increase the palliation time according to the patient's growth is desirable. In recent years, expandable shunts (exGraft™ PECA) have been developed. According to recent material studies, a shunt could increase diameter after endovascular balloon dilatation. In this case report, we describe the first case of endovascular Blalock-Thomas-Taussig shunt (mBT) shunt expansion in a Tetralogy of Fallot / atrial-ventricular Septal Defect complete (TOFAVSDc) patient with trisomy 21 who went to palliative treatment for tracheomalacia (noncardiac lesion association), severe pulmonary arteries hypoplasia, and low weight. This case introduces the "Smart Palliation concept" in the clinical scenario of selected growing patients where the lifetime of the Blalock-Thomas-Taussig (BT) shunt, anatomic substrates, and complexity of clinical status may require an additional palliation time. The limitation of endovascular conduit expansion is the fragility of the anastomosis site. The anastomosis site is a lesser strength structure of the conduit, and dilatation could develop procedure complications. For this reason, in this paper, we introduced our project design: a new technique (Clepsydra Shape) that consists, before surgical implantation, of pre-expansion of the proximal and distal anastomotic parts of the shunt to obtain an increase of 30% in size of both anastomotic sides, preventing stress- and stretch-related lesion of future balloon dilatation.

Impact of hard lockdown on interventional cardiology procedures in congenital heart disease: a survey on behalf of the Italian Society of Congenital Heart Disease.

The Coronavirus disease 2019 (COVID-19) pandemic has thoroughly and deeply affected the provision of healthcare services worldwide. In order to limit the in-hospital infections and to redistribute the healthcare professionals, cardiac percutaneous intervention in Pediatric and Adult Congenital Heart Disease (ACHD) patients were limited to urgent or emergency ones. The aim of this article is to describe the impact of the COVID-19 pandemic on Pediatric and ACHD cath laboratory activity during the so-called 'hard lockdown' in Italy. Eleven out of 12 Italian institutions with a dedicated Invasive Cardiology Unit in Congenital Heart Disease actively participated in the survey. The interventional cardiology activity was reduced by more than 50% in 6 out of 11 centers. Adolescent and ACHD patients suffered the highest rate of reduction. There was an evident discrepancy in the management of the hard lockdown, irrespective of the number of COVID-19 positive cases registered, with a higher reduction in Southern Italy compared with the most affected regions (Lombardy, Piedmont, Veneto and Emilia Romagna). Although the pandemic was brilliantly addressed in most cases, we recognize the necessity for planning new, and hopefully homogeneous, strategies in order to be prepared for an upcoming new outbreak.

A Rare Variant of Hypogenetic Lung Syndrome Mimicking Scimitar Vein.

Hypogenetic lung syndrome, also known as scimitar syndrome, is a rare and well-known congenital condition that includes hypoplastic right pulmonary artery and lung, right displacement of the heart, anomalous systemic arterial supply to the lung, and a characteristically curved anomalous right pulmonary vein draining into the inferior vena cava. In exceptional cases, the anomalous pulmonary vein may drain into left atrium. We here report a case of a 17-year-old girl with a rare variant of hypogenetic lung syndrome diagnosed by means of multimodality imaging and treated with percutaneous occlusion of the aortopulmonary collateral.

Atrial septal defect morphology and stenting in hypoplastic left heart syndrome after hybrid palliation.

Aims The aim of this study was to describe atrial septal defect morphology in hypoplastic left heart syndrome, to report the incidence of restrictiveness and its relationship with defect morphology, to correlate restriction with midterm outcome, and to describe our interventional approach to restrictive defect. Methods and results From 2011 to 2015, 31 neonates with hypoplastic left heart syndrome underwent hybrid procedure with pulmonary artery banding and ductal stenting at our Institution. Restrictive physiology of the atrial septal defect was based on Doppler gradient >6 mmHg through the defect and on clinical signs of pulmonary hypertension. The mean gradient was then measured invasively. Restrictive defect occurred in 11/27 patients (40%). The restrictive group showed three ostium secundum defects (27%) and eight complex morphologies (73%). Conversely, in the non-restrictive group, we observed 11 ostium secundum defects (69%) and five complex morphologies (31%). Early balloon atrioseptostomy was required in three cases. Late restriction occurred in eight patients and was dealt with balloon dilation, stenting, or atrioseptectomy. There was no significant difference between restrictive and non-restrictive groups in terms of early or 12-month survival. CONCLUSIONS: Complex morphologies were more frequently related to restrictiveness. Stenting technique has a crucial role, as the procedure carries a significant risk for stent migration. Effective treatment of restrictive atrial septal defect is related to a better outcome, as it leads to equalisation of survival between patients with and those without restrictive atrial septal defect.

Early hybrid approach and enteral feeding algorithm could reduce the incidence of necrotising enterocolitis in neonates with ductus-dependent systemic circulation.

BACKGROUND: The reported incidence of necrotising enterocolitis in neonates with complex CHD with ductus-dependent systemic circulation ranges from 6.8 to 13% despite surgical treatment; the overall mortality is between 25 and 97%. The incidence of gastrointestinal complications after hybrid palliation for neonates with ductus-dependent systemic circulation still has to be defined, but seems comparable with that following the Norwood procedure. METHODS: We reviewed the incidence of gastrointestinal complications in a series of 42 consecutive neonates with ductus-dependent systemic circulation, who received early hybrid palliation associated with a standardised feeding protocol. RESULTS: The median age and birth weight at the time of surgery were 3 days (with a range from 1 to 10 days) and 3.07 kg (with a range from 1.5 to 4.5 kg), respectively. The median ICU length of stay was 7 days (1-70 days), and the median hospital length of stay was 16 days (6-70 days). The median duration of mechanical ventilation was 3 days. Hospital mortality was 16% (7/42). In the postoperative period, 26% of patients were subjected to early extubation, and all of them received treatment with systemic vasodilatory agents. Feeding was started 6 hours after extubation according to a dedicated feeding protocol. After treatment, none of our patients experienced any grade of necrotising enterocolitis or major gastrointestinal adverse events. CONCLUSIONS: Our experience indicates that the combination of an "early hybrid approach", systemic vasodilator therapy, and dedicated feeding protocol adherence could reduce the incidence of gastrointestinal complications in this group of neonates. Fast weaning from ventilatory support, which represents a part of our treatment strategy, could be associated with low incidence of necrotising enterocolitis.

Risk factors for postoperative delirium after colorectal surgery for carcinoma.

BACKGROUND AND AIM: Data regarding the incidence and risk factors for postoperative delirium (PD) after gastrointestinal surgery are heterogeneous because they include both benign and malignant disease. The aim of this study was to investigate the incidence and risk factors for PD in 100 consecutive patients over 65 years who underwent colorectal surgery for carcinoma. METHODS: Pre-operative cognitive function was assessed using the Mini Mental State Examination. The onset of PD was diagnosed by the Confusion Assessment Methods administered to the patients every 12 h starting from the first postoperative day to discharge. The severity of PD was also evaluated with the Delirium Rating Scale. Different parameters: pre-, intra- and postoperative, were analyzed. Univariate and multivariate analyses were performed. RESULTS: PD developed in 18% of patients. Univariate analysis revealed that advanced age, a history of PD, alcohol abuse, lower blood albumin concentration, intra-operative hypotension, elevated infusion volume and excessive blood loss were significantly related to the development of PD. On multivariate analysis, only lower levels of albumin, alcohol abuse, and hypotension were independent risk factors for PD. CONCLUSIONS: These findings suggest that PD is a frequent complication after colorectal surgery for carcinoma. A model based on pre, intra and postoperative risk factors allows prediction of the patient's risk for developing PD in order to implement preventive measures for this complication.

Stenting of aortic coarctation and exercise-induced hypertension in the young.

OBJECTIVES: To evaluate whether the stenting of aortic coarctation enhance the risk of exercise-induced hypertension (EIH). BACKGROUND: There is the theoretical concern that aortic stents may cause increased aortic wall impedance and therefore systolic hypertension during exercise. METHODS: Blood pressure and the Doppler derived peak and mean systolic pressure gradient (PSG and MSG) across the distal aorta at the peak of exercise were evaluated in young patients (mean age 14 +/- 3 years) with aortic coarctation successfully treated with surgery or with stent implantation at least 1 year before the test. Only patients who reached the 85% maximal predicted heart rate or whose exercise test was interrupted because of severe hypertension, and in whom significant aortic narrowings were excluded by a MRI or a CT scan performed in the six months preceding the exercise test were included in the study. RESULTS: Seventeen patients formed the surgery-group, while 15 patients the stent-group. Patients in surgery-group were younger at coarctation repair and with a longer follow-up than those in stent-group. No difference was present regarding age, body surface area, gender, and presence, and degree of mildly hypoplastic aortic segments between the two groups as well as between patients with or without EIH. EIH was found in 35% of surgery-group patients and in 33% of stent-group patients. PSG and MSG were similar in the patients with or without EIH. CONCLUSIONS: EIH can be found in a high number of young patients successfully treated for aortic coarctation but at intermediate follow-up stent implantation does not seem to enhance the risk of EIH.