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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., (Copyright © 2021 Italian Federation of Cardiology - I.F.C. All rights reserved.)

Anomalous drainage of the inferior vena cava into the left atrium is a rare congenital condition. A 20-year-old girl was referred for recurrent transient ischemic attacks. Transthoracic echocardiography revealed a large ostium secundum atrial septal defect, and computed tomography showed anomalous drainage of the inferior vena cava into the left atrium. Through a right mini-thoracotomy, the opening of the inferior vena cava into the atrium was identified under the inferior edge of the interatrial septum, draining into the left atrium, and redirected to the right atrium, using a pericardial patch to reconstruct the atrial septum. Postoperative course was uneventful. Right mini-thoracotomy approach was effective in correcting the anomalous drainage of the inferior vena cava into the left atrium.

Background: The arterial switch operation (ASO) for transposition of the great arteries requires transfer of the coronary arteries from the aorta to the proximal pulmonary artery (neoaorta). This is complicated by variable coronary anatomy before transfer. In 8% to 10% of cases, there is evidence of late coronary stenosis and/or occlusion, often with catastrophic clinical consequences. The mechanism of such complications has not been well studied., Methods and Results: We analyzed 190 consecutive high-resolution computed tomographic scans from the ASO procedure (patients aged 5-16 years) and found 17 patients with significant (>30% up to occlusion) coronary lesions (8.9%); all were later confirmed by conventional angiography. The left main coronary artery was abnormal in 9 patients (ostium in all), the left anterior descending artery in 3, the circumflex in 2, and the right coronary artery in 3 patients. Using multiplanar and 3-dimensional reconstructions of the coronary arteries, aorta, and pulmonary artery, we identified the commonest mechanisms of coronary abnormalities. For the left main and left anterior descending artery, anterior positioning of the transferred left coronary artery (between 12 and 1 o'clock on the neoaorta) appeared to predispose to a tangential course of the proximal left coronary artery promoting stenosis. All circumflex lesions occurred in Yacoub type D coronaries where a long initially retroaortic artery was stretched by its new positioning behind an enlarged neoaorta. Right coronary artery lesions occurred only in cases in which the reimplantation site was very high above the right coronary sinus with potential compression from the main pulmonary artery bifurcation immediately above., Conclusions: Thus detailed multiplanar computed tomographic scanning can elucidate the mechanisms of late coronary complications after the ASO. Understanding these aspects could help to improve surgical technique to minimize the risk of late coronary obstructions., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

We report the case of a child with severe and atypical stenosis of the left main coronary artery, which occurred late after arterial switch operation for transposition of the great arteries. Cardiac computed tomography accurately defined the lesion, showing the presence of post-stenotic dilation, guided the surgical approach and assessed coronary patency after revascularisation surgery.

Congenital mitral regurgitation is rare and usually part of complex cardiac anomalies. When needed, early surgery represents a great challenge. In small babies avoiding valve replacement is desirable, but valve repair may be extremely complex. We describe an isolated congenital mitral regurgitation, successfully treated with conservative surgery about 1 h after birth. In a 30-year-old pregnant woman, fetal echocardiography revealed mitral annular dilatation with massive regurgitation, functional aortic atresia and a very small patent foramen ovale. Realizing that the baby had a poor chance of survival after birth, a cesarean section was scheduled at 37 weeks of pregnancy. The procedure was performed in the operating room next to the cardiac surgery theatre, where the newborn was urgently transferred. After an unsuccessful attempt of percutaneous atrial septostomy, a rescue surgical mitral repair was performed. To avoid mitral replacement, moderate residual regurgitation was accepted. Postoperative hospital stay was 57 days and the baby was discharged in good clinical condition. Residual mitral regurgitation was moderate at discharge and decreased thereafter. During a five-year follow-up the child remained asymptomatic with normal growth. Preserved ventricular function and progressive volume reduction of left heart chambers were observed.

Objectives: We investigated the accuracy of 64-slice computed tomography (CT) angiography, as compared to invasive angiography, to evaluate reimplanted coronary arteries in children after arterial switch operation (ASO) for transposition of the great arteries (TGA)., Background: Assessment of the integrity of reimplanted coronary arteries is crucial for long-term outcome after ASO for TGA. Noninvasive tests have limited accuracy for detecting significant coronary lesions, and invasive coronary angiography is usually required in this setting., Methods: One hundred thirty consecutive children, after ASO for TGA (age 5.6 +/- 1.1 years), underwent conventional invasive coronary angiography and coronary CT angiography using a 64-slice scanner. The ability of CT to detect significant coronary stenoses (>30% diameter reduction) of the coronary ostia and proximal segments, and other abnormalities of the coronary arteries was analyzed by blinded comparison to the invasive coronary angiogram., Results: The CT was fully evaluable in 126 of 130 patients (97%), allowing assessment of ostia and proximal segments of all coronary arteries. The CT correctly detected all 12 patients (9.2%) in whom invasive coronary angiography had identified significant coronary lesions, with a sensitivity, specificity, and negative predictive value of 100%. In addition, CT showed nonsignificant coronary lesions (<30% luminal narrowing) in 6 patients and allowed determination of the underlying reasons for coronary luminal narrowing, such as stretching or compression of the re-implanted coronary arteries caused by their anatomic relationship to the adjacent great vessels., Conclusions: 64-slice CT coronary angiography performs as well as invasive angiography for detecting significant coronary lesions in the majority of children who have undergone the arterial switch procedure for TGA. CT also provides information on the underlying mechanism of coronary luminal narrowing.

Objective: We assessed the contribution of acute aortic arch angulation and enhanced systolic pulse wave reflection to dilatation of the ascending aorta and aortic regurgitation late after the arterial switch operation for transposition of the great arteries., Methods: We performed aortography, radial artery applanation tonometry, and transthoracic echocardiography in 47 children (aged 5-6 years) who underwent the arterial switch operation and in 20 matched healthy controls. The aortic arch angle, ratio of ascending/descending aortic diameter, degree of aortic regurgitation, central pulse pressure, aortic augmentation pressure, and augmentation index were measured., Results: The aortic arch angle was more acute (55 +/- 6.5 degrees vs 68 +/- 5 degrees, respectively, P < .001) and the ratio of the ascending/descending aorta diameter was significantly greater (1.98 +/- 0.4 vs 1.55 +/- 0.06, respectively, P < .001) in the patients who underwent the arterial switch operation compared with controls. Augmentation pressure and augmentation index were higher in the patients who underwent the arterial switch operation than in controls (7.5 +/- 4.6 vs 3.4 +/- 5.8, respectively P = .04; 21 +/- 10 vs 8 +/- 13, respectively, P = .005). A more acute aortic angle was associated with a higher aortic augmentation index (r = 0.41, P < .01), a greater ratio of the ascending to descending aorta (r = -0.6, P < .001), and the degree of aortic regurgitation (r = 0.39, P < .01)., Conclusion: Sharper angulation of the aortic arch is associated with early pulse wave reflection, dilatation of the ascending aorta, and aortic regurgitation late after the arterial switch operation for transposition of the great arteries.

Background: The objective of this study was to review our institutional experience concerning neonatal aortic commissurotomy and to identify predictors of outcome., Methods: From January 1, 1990, to January 1, 2005, 65 neonates were referred for critical aortic stenosis. Among these 36 consecutive patients underwent surgical valvotomy by the same surgeon and 16 patients had balloon valvotomy. We examined clinical records to establish determinants of outcome and illustrate long-term results., Results: In the surgical group 6 patients had nonstenotic parachute mitral valve, 6 had aortic coarctation, 13 had endocardial fibroelastosis; and 15 had depressed fractional shortening. In the balloon valvotomy group most infants had associated mitral stenosis (n = 7), small aortic annulus (n = 5), multiple anomalies (n = 2), endocardial fibroelastosis (n = 9), and depressed fractional shortening (n = 13). In the surgical group global mortality was 19%; 47% in infants with depressed fractional shortening and 0% in infants with normal fractional shortening. Mortality in the balloon valvotomy group was 56%. Low fractional shortening, low mean aortic gradient, endocardial fibroelastosis, and small aortic annulus were associated with fatal outcome. In the surgical group 6 patients needed Ross operation at a median interval of 4 years from commissurotomy. In the balloon valvotomy group 3 patients needed early mitral replacement and 5 aortic valve surgery. At a median follow-up of 6 years, all children are in New York Heart Association class I, all have a normal ventricular function, 2 have aortic restenosis, and 5 have at least moderate aortic incompetence., Conclusions: In a selected population of neonates with isolated aortic stenosis, surgical valvotomy offers an excellent long-term outcome to patients with a normokinetic left ventricle, while patients with a hypokinetic left ventricle have a poor outcome. Balloon valvotomy offers poor results when performed in patients with complex anomalies.

Objective: To study the effects of closure of interatrial communications associated with a right to left shunt in patients with congenital heart disease (CHD) who had a biventricular repair., Design: Retrospective study., Setting: Tertiary referral centre., Patients: 15 patients with CHD with right to left shunt through an interatrial communication: three had repaired tetralogy of Fallot, five had repaired pulmonary atresia with intact ventricular septum, four had Ebstein's disease, and three had other CHDs. Two patients had had a stroke before closure of the interatrial communication., Interventions: Percutaneous atrial septal defect (n = 6) or persistent foramen ovale (n = 9) closure. All patients underwent an exercise test before and after interatrial communication closure., Results: Five patients were cyanotic at rest. During exercise, mean (SD) oxygen saturation diminished from 93.9 (3.8)% to 84.3 (4.8)% (p < 0.05). Interatrial communication closure led to an immediate increase of oxygen saturation from 93.9 (3.8)% to 98.6 (1.6)% (p < 0.05). At a median follow up of three years (range 0.5-5) all but one patient with a residual atrial septal defect had normal oxygen saturation at rest and during exercise. Maximum workload increased from 7.2 (1.9) to 9.0 (2.2) metabolic equivalents (p < 0.001)., Conclusions: Percutaneous closure of interatrial communications associated with a right to left shunt allows restoration of normal oxygen saturation at rest, avoidance of desaturation during exercise, and improvement of exercise performance in patients with CHD.

Obstructive sleep apnoea (OSA) is a common sleep disorder often associated with cardiovascular problems. The role of right to left shunt through a patent foramen ovale (PFO) in OSA is still debated. We report the case of a 42-year-old man with OSA, treated by nocturnal continuous airway pressure, who had a PFO and severe dispnoea and desaturation at exercise. Percutaneous closure of PFO leads to immediate and complete resolution of symptoms. Our report shows for the first time that patients with OSA, PFO, and desaturation at exercise should be considered candidates to PFO closure, even in the absence of cerebral events., ((J Interven Cardiol 2005;18:393-395).)

Objective: We evaluate early results of bovine jugular vein conduits in the pulmonary outflow., Methods: Between April 2000 and September 2001, 31 conduits were placed in the outflow of the right ventricle. Patients who received a conduit as a staged surgical procedure were excluded (n = 3). Implantation age ranged from 0 to 21 years (median, 3.4 years). Conduit diameter ranged from 12 to 20 mm (median, 14 mm). Transthoracic echocardiography was performed at discharge and 3 months after surgery. Patients with significant pulmonary regurgitation and/or stenosis underwent cardiac catheterization., Results: Four patients died during the follow-up period. Three deaths were unrelated to the conduit. One death was related to the complete thrombosis of the conduit. At 3 months evaluation, pulmonary valve regurgitation was absent or trivial in 19, mild in 2 and severe in 3 of 24 survivors. Four patients had nonfatal conduit-related complications. A transient thrombus formation within 1 leaflet was noted postoperatively in a patient with a moderate pulmonary regurgitation. Three patients required reoperation 3 to 5.8 months after the implantation for conduit failure (mean, 4.3 months). Cardiac catheterization before replacement revealed an aneurysmal dilation of the conduit below a severe stenosis of the pulmonary bifurcation due to important neointimal proliferation., Conclusions: Early failure of bovine jugular vein valved conduits can occur because of exaggerated intimal proliferation or thrombotic process within the conduit. Because of these complications, close echocardiographic follow-up is mandatory during the first weeks after implantation.

Objectives: We evaluated the long-term results of perforation of the pulmonary valve in patients with pulmonary atresia with an intact ventricular septum (PA-IVS)., Background: Interventional perforation of the pulmonary valve is considered the elective first stage treatment for PA-IVS, particularly in patients with a tripartite right ventricle (RV) and normal coronary circulation. However, the long-term results of this procedure are lacking., Methods: Between January 1991 and December 2001, 39 newborns with a favorable form of PA-IVS underwent attempted perforation of the pulmonary valve. We evaluated the early and long-term outcomes., Results: Median tricuspid and pulmonary z values were -1.2 and -2.4, respectively. Perforation was successful in 33 patients. Among them, 17 needed neonatal surgery, 13 did not need any surgery, and 3 had elective surgery after the first month of life. There were two procedure-related deaths, seven nonfatal procedural complications, and four postsurgical deaths. Compared with patients needing neonatal surgery, those having no or elective surgery had a higher incidence of a tripartite RV and a higher median tricuspid z value (92% vs. 53%, p = 0.04 and -1.7 vs. -0.5, p = 0.03). At a median follow-up of 5.5 years (range 0.5 to 11.5), survival was 85% and freedom from surgery was 35%. Five patients, four of whom had neonatal surgery, underwent a partial cavo-pulmonary connection., Conclusions: Our results show that this technique, although burdened by non-negligible mortality and morbidity, is effective in selected patients with a normal-sized RV. Preselection of patients allows interventional or surgical biventricular correction in the majority of cases.

Although the etiology of pulmonary fistulas in congenital heart disease remains unknown, several hemodynamic factors are thought to play a role. Recently it was postulated that hepatic venous effluent might protect the pulmonary bed from development of pulmonary fistulas. However, there are no experimental data supporting this hypothesis. Here, we present the cases of 2 patients in whom pulmonary fistulas developed after unintentional surgical exclusion of the hepatic venous flow from the pulmonary circulation. In both patients, symptoms resolved after rerouting of the hepatic venous blood to the lungs.