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.

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.

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.

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.

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.

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.

Atrial flow regulator for failing Fontan circulation: an initial European experience.

Transcatheter creation or enlargement of an atrial septal defect has been used to promote adequate blood flow and mixing in some forms of congenital heart defects or as a relief valve in right or left atrial hypertension, resulting in better cardiac output and/or systemic saturation. We report a case of a 4-year-old male affected by complex congenital heart disease who was admitted for management of severe cyanosis following a staged pericardial fenestrated Fontan procedure. Transoesophageal echocardiogram showed a wide fenestration of 9 mm in size with a severely dilated pericardial Fontan system. To avoid a new surgical procedure and as part of a compassionate use programme, we decided to implant an atrial flow regulator device (4 mm in diameter) with percutaneous approach with the goal of reducing the right-to-left shunt and increasing the pulmonary flow. Preprocedural oxygen saturation was 75%, whereas after 2 months of follow-up, we observed a progressive increase of up to 95% with significant reduction in the pericardial Fontan system dimensions at echocardiography.

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.

Double aortic arch with hypoplastic right aortic arch and type C atresia of left aortic arch.

We report the case of a 2-month-old baby with a double aortic arch, type C atresia of the left arch and severe hypoplasia of the right aortic arch between the right carotid and subclavian arteries, resulting in systemic obstruction, left ventricular dysfunction and congestive heart failure. Surgical augmentation of the right aortic arch ameliorated the obstruction with improvement in left ventricular function and symptoms.

Bidirectional Glenn and antegrade pulmonary blood flow: temporary or definitive palliation?

BACKGROUND: We sought to investigate the role of the bidirectional Glenn with antegrade pulmonary blood flow in the surgical history of children with univentricular hearts. METHODS: A series of 246 patients, from three joint institutions, having univentricular heart with restricted but not critical pulmonary blood flow received a bidirectional cavopulmonary shunt with additional forward pulmonary blood flow. All patients have been studied according to their progression, or not, to Fontan operation. Two hundred and eight (84.5%) patients underwent bidirectional cavopulmonary anastomosis as primary palliation. Twenty patients (8.1%) with previous pulmonary artery banding were also enrolled in the study. Patients who had received additional pulmonary blood flow through a previous systemic to pulmonary artery shunt for the critical pulmonary blood flow were excluded. RESULTS: No in-hospital death occurred. Follow-up was complete at 100%. Mean follow-up was 4.2 +/- 2.8 years (range, 6 months to 7 years). During the observational period 73 (29.7%) patients, considered optimal candidates, underwent Fontan completion for increasing cyanosis and (or) hematocrit and (or) fatigue with exertion. Three patients expired after total cavopulmonary connection (3 of 73; 4.1% mortality rate). The remaining 173 (70.3%) patients are alive with initial palliation. All patients were still well palliated with an arterial oxygen saturation at rest about 90%. CONCLUSIONS: According to our experience and results, bidirectional Glenn with antegrade pulmonary blood flow may be an excellent temporary palliation prior to a Fontan operation, which can be performed at the onset of symptoms. Bidirectional Glenn may also be the best possible palliation for a suboptimal candidate for Fontan.

Extracorporeal circulation, optimized: a pilot study.

We designed a pilot study to assess as primary end point the safety and efficacy of a new phosphorylcholine-coated, closed cardiopulmonary bypass (CPB) system (extracorporeal circulation, optimized [ECC.O], Dideco, Mirandola, Italy). The secondary end point was to compare results with two retrospectively matched cohorts of patients who underwent isolated coronary artery by-pass graft (CAGB) with nonphosphorylcholine-bonded circuits and cardiotomy suction (Group II, n = 32) and off-pump coronary artery by-pass (OPCAB) (Group III, n = 26). In January 2005, 30 patients (Group I) undergoing first-time CABG were assigned to the ECC.O group. Five minutes after CPB, initial hematocrit levels were significantly and consistently highest in Group I relative to Group II (Group I, 29.7 +/- 4.4 vs. Group II, 22.7 +/- 4.1; P < 0.001). Red blood cell transfusion rate was reduced drastically in Group I versus Group II (P < 0.001). High differences were also observed in C-reactive protein levels at 24 h after surgery (Group I vs. Group II-P < 0.001 and vs. Group III-P < 0.001) and at 72-h peak value (Group I vs. Group II-P < 0.001 and vs. Group III-P < 0.001). The routine clinical use of the ECC.O system has been demonstrated to be both clinically safe and efficacious. An intensive training program for surgeons, perfusionists, and anesthesiologists is required.

DIDECMO: a new polymethylpentene oxygenator for pediatric extracorporeal membrane oxygenation.

We reviewed the performance of a new polymethylpentene oxygenator (DIDECMO, Dideco, Mirandola, Italy) in terms of clinical safety and efficiency in priming, oxygenation, and oxygenator resistance in neonatal and pediatric extracorporeal membrane oxygenation (ECMO) patients. Between March 2005 and January 2006, 14 patients required ECMO in the San Vincenzo Hospital. Of these, 8 (median age, 9 days; range, 3 days to 15 months) received normothermic ECMO for postcardiotomy heart failure after surgery for congenital heart disease. The DIDECMO oxygenator was used in all patients (median weight, 2.4 kg; range, 2 to 7 kg). According to our previous experience, all patients received the same anticoagulation management. DIDECMO is a new phosphorylcholine-coated, polymethylpentene hollow-fiber oxygenator recommended for a maximum blood flow of 2300 ml/min with a membrane surface area of 0.67 m2 and validated to be used up to 5 days. Static priming was 100 ml and mean support time 05 hours (range, 36 to 198 hours). No oxygenators were changed during support. Median pressure drop during overall assistance was 24 mm Hg. Carbon dioxide elimination was obtained with a 1:1 blood flow/air flow ratio. Neither oxygenator-related major nor minor adverse events occurred during support. In our initial experience, the new polymethylpentene DIDECMO oxygenator provided adequate gas exchange and offered technical advantages in terms of low priming volume and acceptable hemodynamic resistance despite pulsatile flow regimen. Also, we used this device for more than 8 days without any technical problems.

Use of a novel anticoagulation strategy during ECMO in a pediatric population: single-center experience.

We describe a novel anticoagulation strategy with continuous intravenous antithrombin infusion and intermittent heparin infusion in pediatric population during extracorporeal membrane oxygenation (ECMO). From November 2004 through February 2006, 11 patients required ECMO for postcardiotomy cardiorespiratory failure. The mean duration of support time was 112 hours (range 68-192 hours). Since April 2005, we modified our anticoagulation protocol in the last six patients. Continuous antithrombin infusion was started immediately after surgery based on the lab result. The antithrombin level was maintained >100% using the following formula: 100 (target value) - (Antithrombin value on lab test) x weight in 4 hours. Antithrombin value was checked at 4-hour intervals. Heparin infusion was started when the antithrombin value was > 100% and remained stable for more than 12 hours and the amount of bleeding was < 2 ml/kg for more than 3 consecutive hours; then heparin infusion was started at 2 UI/kg/h via the oxygenator (target ACT was not < 150 seconds). Three patients in the first group died. Eight patients were weaned and discharged; the third, fourth, and fifth required surgical revision for bleeding. One experienced minor neurologic sequelae. Neither surgical revision nor thromboembolic complications occurred in the new anticoagulation group. A novel anticoagulation strategy utilizing continuous intravenous antithrombin and intermittent heparin infusion reduced significantly surgical revision for bleeding in the first 48 hours. This has translated into excellent overall outcomes.