Extracorporeal Membrane Oxygenation (ECMO) in an Infant with COVID-19: A Case Report with Literature Review.

BACKGROUND: SARS-CoV-2 infection tends to be lethal to the elderly population. However, sometimes children are also involved. CASE PRESENTATION: We present the case of a female infant with a corrected gestational age of 39 weeks and 4 days with severe COVID-19 pneumonia and co-infection of Klebsiella pneumoniae that was supported with extracorporeal membrane oxygenation (ECMO). RESULTS: We reported the clinical case and reviewed the literature articles on ECMO and Covid-19 in infants and children up to two years of age. CONCLUSION: It is crucial to be aware of certain risk factors (severe prematurity, coinfection), which, when linked to SARS-CoV-2 infection, must immediately alert us to the possible criticality of the clinical condition of patients, as highlighted by our own clinical case.

Global Cardiac Surgery-Accessibility to Cardiac Surgery in Developing Countries: Objectives, Challenges, and Solutions.

Cardiac surgery is a modern science in the history of medicine. The impact of cardiac disease, in terms of treatment and prognosis, has made this discipline indispensable to global health. In recent decades, the greatest investment has been dispensed to technological and material improvements to increase life expectancy. This surgery must address different epidemiological aspects dictated by the geography and economic-social conditions of the global populations. For this reason, it is progressively important to address the cardiac surgery accessibility disparity. Many scientific papers and international meetings have studied how cardiac surgery can be more accessible in various countries around the world. In this review, we analyze all the challenges, solutions, and suggestions that can make this surgery accessible to the entire global population, with the purpose of reducing its disparity across all seven continents. For a long time, high-income countries have invested in technological capabilities and experimental advancements without caring about unequal access in the rest of the world. We believe that it is time to reverse this growth trajectory, placing the accessibility and distribution of surgical science as a priority, which is significant for the right to health of all people worldwide. This is the real new challenge in cardiosurgery.

Anomalous venoatrial connections - CT and MRI assessment.

Abnormal venous atrial (VA) connections present a congenital heart disease (CHD) challenge for pediatric cardiologists. Fully anatomical evaluation is very difficult in prenatal and perinatal follow-up, but it has a profound impact on surgical correction and outcome. The echocardiogram is first-line imaging and represents the gold standard tool for simple abnormal VA connection. CT and MRI are mandatory for more complex heart disease and "nightmare cases". 3D post-processing of volumetric CT and MRI acquisition helps to clarify anatomical relationships and allows for the creation of 3D printing models that can become crucial in customizing surgical strategy. Our article describes a ten-year (2013-2022) tertiary referral CHD center of abnormal AV connections investigated with CT and MRI, illustrating most of these complex diseases with the help of volume rendering (VR) or multiplanar reconstructions (MPR). The nightmarish cases will also be addressed due to the complex cardiovascular arrangement that requires a challenging surgical solution for correction along with the post-surgical complications.

Hybrid Palliation for Hypoplastic Left Heart Syndrome: Role of Echocardiography.

Hypoplastic left heart syndrome is a spectrum of complex congenital cardiac defects. Although in borderline cases, biventricular repair is a viable option, in the majority of cases, univentricular palliation is the treatment of choice. Hybrid palliation can be a valid alternative to classic Norwood operation in the neonatal period, especially in selected cases such as high-risk patients or borderline left ventricles. Echocardiography is the main diagnostic modality in this pediatric population, from the fetal diagnosis to the subsequent surgical steps of palliative treatment. Hybrid palliation is performed after birth and is characterized by surgical banding of the pulmonary arteries along with transcatheter stenting of the ductus arteriosus. There are some peculiar aspects of cardiac imaging that characterize this type of palliation, and that should be considered in the different phases before and after the procedure. We aimed to review the current literature about the role of echocardiography in the management of patients with hypoplastic left heart undergoing hybrid palliation.

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.

Diagnosis and Management of Button Battery Ingestion Complicated by Tracheo-Esophageal and Aorto-Esophageal Fistulas.

Button battery ingestion (BBI) is common in children and its prevalence has increased in the last decades. BBI can be responsible for very severe and potentially fatal complications if not promptly detected. We describe the successful management of two cases of BBI that occurred in two previously healthy infants. Both patients presented with vague symptoms and no witness of foreign body ingestion. The prolonged time of exposure to the corrosive effects of disk batteries was responsible for the development of tracheo-esophageal fistula (TEF) and aorto-esophageal fistula (AEF). We demonstrate how prompt diagnosis and management are crucial for the infants' survival.

Anatomically corrected malposition of the great arteries (S,L,D) with mutation of Nodal gene.

Anatomically corrected malposition of the great arteries is a rare CHD, involving alignment and position of the great arteries. We report an infant with situs solitus, atrioventricular discordance, and ventriculoarterial concordance with the aorta arising anteriorly and to the right of the pulmonary artery. A mutation of Nodal gene, implicated in the pathogenesis of human left-right patterning defects, was found.

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 COVID-19 Pandemic on the Italian Humanitarian Congenital Cardiac Surgery Activity: What No One Tells You.

More than 4 millions of children with congenital heart disease (CHD) are waiting for cardiac surgery around the world. Few of these patients are treated only thanks to the support of many non-governmental organizations (NGOs). Starting in December 2019, the so-called coronavirus disease 2019 (COVID-19) has rapidly become a worldwide pandemic and has dramatically impacted on all the international humanitarian activities for congenital heart disease. We analyzed data from all the Italian congenital cardiac surgery centers with the aim to quantify the impact of the pandemic on their charities. Fifteen Italian centers participated in the study and contributed to data collection. We analyzed and compared data regarding humanitarian activities carried out abroad and on site from two periods: year 2019 (pre-COVID-19) and year 2020 (COVID-19 pandemic). In 2019, 53 international missions were carried out by Italian congenital cardiac surgeons, resulting in the treatment of 471 CHD patients. In the same period 11 Italian cardiac centers operated on 251 foreign patients in Italy. In 2020, the pandemic led to a reduction of this activity by 96% for the surgery performed overseas and 86% for the interventions carried out in Italy. In conclusion our study shows the important quantitative impact of the pandemic on the Italian humanitarian cardiac surgical activity overseas and in Italy. This shocking result highlights the failure of the systems adopted so far to solve the problem of CHD in developing countries.

Tracheal trifurcation: new cases and review of the literature.

BACKGROUND: Tracheal trifurcation is an uncommon and often unknown type of anomalous tracheobronchial arborization, characterized by three main bronchi originating at the level of the carina. Diagnosis is important due to its clinical implications. OBJECTIVE: To highlight the anatomical, clinical and diagnostic aspects of tracheal trifurcation by reporting our experience and reviewing the literature. MATERIALS AND METHODS: We retrospectively evaluated pediatric patients referred to our institution from January 2018 to May 2020 with a diagnosis of tracheal trifurcation. All patients underwent chest radiographs, computed tomography (CT) (with/without dynamic airway scanning) and bronchoscopy. Clinical and anatomical data were collected. RESULTS: Three patients with tracheal trifurcation were identified (1 female, median age: 7.6±0.4 months). All had associated anomalies: two had tetralogy of Fallot, one with esophageal atresia/tracheoesophageal fistula and one with an atrioventricular septal defect, and the other had skeletal ciliopathy. Chest radiographs were not diagnostic for tracheal trifurcation. Bronchoscopy could not fully evaluate the trachea and main bronchi in two cases. CT detected tracheal trifurcation in all of the patients and also demonstrated other lung parenchymal and congenital anomalies. Two of the three main bronchi were directed to the right lung in all cases. Ostial stenosis of either the central (two patients) or right upper bronchus (one patient) was present. No signs of tracheobronchomalacia were found. CONCLUSION: Tracheal trifurcation is rare and often associated with additional disorders, such as other tracheobronchial anomalies, cardiovascular defects or esophageal malformations, responsible for clinical manifestations and earlier detection. Bronchoscopy is often used for diagnosis, but is invasive and may be incomplete or inconclusive, while CT allows for a noninvasive and correct diagnosis, while also highlighting additional findings in the thorax.

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.

Impact of the coronavirus disease 2019 (COVID-19) pandemic on the Italian congenital cardiac surgery system: a national survey.

OBJECTIVES: Italy has been one of the countries most severely affected by the coronavirus disease 2019 (COVID-19). The Italian government was forced to introduce quarantine measures quickly, and all elective health services were stopped or postponed. This emergency has dramatically changed the management of paediatric and adult patients with congenital heart disease. We analysed data from 14 Italian congenital cardiac surgery centres during lockdown, focusing on the impact of the pandemic on surgical activity, patients and healthcare providers and resource allocation. METHODS: Fourteen centres participated in this study. The period analysed was from 9 March to 4 May. We collected data on the involvement of the hospitals in the treatment of patients with COVID-19 and on limitations on regular activity and on the contagion among patients and healthcare providers. RESULTS: Four hospitals (29%) remained COVID-19 free, whereas 10 had a 39% reduction in the number of beds for surgical patients, especially in the northern area. Two hundred sixty-three surgical procedures were performed: 20% elective, 62% urgent, 10% emergency and 3% life-saving. Hospital mortality was 0.4%. Compared to 2019, the reduction in surgical activity was 52%. No patients operated on had positive test results before surgery for severe acute respiratory syndrome coronavirus 2, the virus responsible for COVID-19. Three patients were infected during the postoperative period. Twenty-nine nurses and 12 doctors were infected. Overall, 80% of our infected healthcare providers were in northern centres. CONCLUSIONS: Our study shows that the pandemic had a different impact on the various Italian congenital cardiac surgery centres based on the different patterns of spread of the virus across the country. During the lockdown, the system was able to satisfy all emergency clinical needs with excellent results.

Atrioventricular Discordance with Double-Outlet Right Ventricle in Mirror Imagery and Levocardia: A Very Rare Case Report.

A newborn without prenatal diagnosis, with bronchial and abdominal situs inversus in levocardia, was referred to our hospital for accurate evaluation; echocardiography showed venoatrial connections in mirror-image arrangement, atrioventricular (AV) discordance, and double-outlet right ventricle (DORV). Additional cardiac malformations were double upper caval district, atrial communication, subpulmonary interventricular communication, and moderate subvalvular and valvular pulmonary stenosis. Few days after birth, the patient presented low oxygen saturation and the heart team decided for a palliative surgery. We describe a very rare case in a newborn with bronchial-abdominal mirror imagery, AV discordance, and DORV in levocardia.

The role of primary surgical repair technique on late outcomes of Tetralogy of Fallot: a multicentre study.

OBJECTIVES: Repair of Tetralogy of Fallot (TOF) has currently excellent results with either transventricular or transatrial approach. However, it is unclear as to which has better late outcomes and what role of residual pulmonary valve (PV) regurgitation in the long term is. We report on late clinical outcomes after repair in a large series of patients with TOF, focusing on the type of surgical technique. METHODS: This analysis is a retrospective multicentre study on patients undergoing TOF repair in infancy. The exclusion criteria of the study were TOF with pulmonary atresia or absent PV. RESULTS: We selected 720 patients who had undergone TOF repair (median age 5.7 months, interquartile range 3.7-11.7). Preoperative cyanotic spells occurred in 18%. A transatrial repair was performed in 433 (60.1%) patients. The PV was preserved in 249 (35%) patients, while the right ventricular outflow tract was reconstructed with a transannular patch (60.4%) or a conduit (4.6%) in the rest of the patients. At a median follow-up of 4 years (range 1-21, 86% complete), 10 (1.6%) patients died, while 39 (6.3%) patients required surgical reoperation and 72 (11.7%) patients required an interventional procedure. The propensity match analysis showed that the incidence of postoperative complications and adverse events at follow-up were significantly increased in patients undergoing transventricular approach repair with transannular patch (P = 0.006) and PV preservation was a significant protective factor against postoperative complications (P = 0.009, odds ratio 0.5) and late adverse events (P = 0.022). CONCLUSIONS: Surgical repair of TOF in infancy is a safe procedure, with good late clinical outcomes. However, transatrial approach and PV preservation at repair are associated with lower early and late morbidity.

Anomalous aortic origin of the pulmonary arteries: Case series and literature review.

Anomalous origin of the pulmonary arteries from the ascending aorta is a rare, but severe clinical entity necessitating a scrupulous evaluation. Either the right or the left pulmonary arteries can arise directly from the ascending aorta while the other pulmonary artery retains its origin from the right ventricular outflow tract. Such a finding can be isolated or can coexist with several congenital heart lesions. Direct intrapericardial aortic origin, however, must be distinguished with origin through a persistently patent arterial duct. In the current era, clinical manifestations usually become evident in the newborn rather than during infancy, as used to be the case. They include respiratory distress or congestive heart failure due to increased pulmonary flow and poor feeding. The rate of survival has now increased due to early diagnosis and prompt surgical repair, should now be expected to be at least 95%. We have treated four neonates with this lesion over the past 7 years, all of whom survived surgical repair. Right ventricular systolic pressure was significantly decreased at follow-up. Our choice of treatment was to translocate the anomalous pulmonary artery in end-to-side fashion to the pulmonary trunk. Our aim in this report is to update an Italian experience in the diagnosis and treatment of anomalous direct origin of one pulmonary artery from the aorta, adding considerations on the lessons learned from our most recent review of the salient literature.

Perfusion Study Helps in the Management of the Intraseptal Course of an Anomalous Coronary Artery.

Anomalous origin of a coronary artery from the opposite aortic sinus of Valsalva can present in various ways, ranging from a benign and incidental finding to sudden cardiac death. The variant with an intraseptal subpulmonary course (sometimes referred to as intraconal), is widely perceived to carry a low risk of ischemia and has been considered to be a benign variant, not requiring surgical treatment. In one of our recent patients, however, nuclear scintigraphy highlighted a myocardial perfusion deficit in the territory supplied by the allegedly benign anomalous coronary artery, prompting the need for a more aggressive surgical approach.