Infradiaphragmatic partial anomalous pulmonary venous connection in adulthood. Considerations for management: a single centre experience.

BACKGROUND AND OBJECTIVES: Infradiaphragmatic partial anomalous pulmonary venous connection is occasionally diagnosed in adulthood. Management of infradiaphragmatic PAPVC depends on anatomy and clinical presentation. METHODS: Over a 10-year period, we observed seven adult patients (median age 29 years) with partial anomalous pulmonary venous connection. We classified our patients in two groups. Group I: isolated partial anomalous pulmonary venous connection from one pulmonary lobe to the inferior vena cava, three patients. Group II: partial anomalous pulmonary venous connection of the entire right lung to IVC, four patients. RESULTS: The mean term follow-up was 5.4 years. Patients in Group I have been managed conservatively, as they were asymptomatic, without a significant shunt. Patients in Group II were surgically corrected using long right intra-atrial baffles. After 6 months of follow-up, the first two cases were diagnosed with complete tunnel thrombosis and loss of right lung function. Oral anticoagulation failed to recanalize the tunnel. Considering this serious complication, the other two patients were empirically and preventively treated with anticoagulation after surgery, with good outcome on long-term follow-up. CONCLUSIONS: Conservative management should be considered for asymptomatic patients, without a significant shunt. Surgical treatment of infradiaphragmatic partial anomalous pulmonary venous connection of the entire right lung in inferior vena cava is challenging. Slow blood flow inside the long intra-atrial baffles inclines to thrombosis and occlusion, as we observed in two cases. Therefore, oral anticoagulation should be considered for long baffles with slow blood flow.

Aortic arch reconstruction in neonates using an autologous pericardial patch.

This video tutorial illustrates the surgical correction of aortic coarctation associated with severe hypoplastic aortic arch and a large subaortic ventricular septal defect using a fresh autologous pericardial patch for arch reconstruction under hypothermic cardiopulmonary bypass and selective cerebral perfusion.

[Anomalous origin of the left coronary artery from the pulmonary artery treated with Takeuchi repair in early childhood. Long-term follow-up of two cases complicated by supravalvular pulmonary stenosis]. / Origine anomala della coronaria sinistra dall'arteria polmonare trattata con tecnica di Takeuchi nella prima infanzia. Follow-up a lungo termine di due casi clinici complicati da stenosi polmonare sopravalvolare.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but serious congenital heart defect. We report two cases of ALCAPA diagnosed in infancy, presenting with low body weight and severe symptoms of congestive heart failure. Both were repaired surgically using Takeuchi technique. Both cases have developed supravalvular pulmonary stenosis at long-term follow-up.

Working as a team in airway surgery: History, present and perspectives.

Teamwork is one of the most important trend in modern medicine. Airway team were created in many places to respond in a multidisciplinary and coordinated way to challenging clinical problems which were beyond the possibility of an individual management. In this chapter, we illustrate the historical steps leading to the development of an airway team in a pediatric referral hospital, describe the present teamwork activity defining the key points for the creation of a team and discussing different organization models; finally we delineate possible future directions for the airway teams in the globalized world.

Procalcitonin and Early Postoperative Infection After Pediatric Cardiopulmonary Bypass Surgery.

OBJECTIVES: Systemic inflammation and bacterial infections are critical occurrences after pediatric cardiac surgery. Elevated white blood cell count and C-reactive protein cannot discriminate between these two conditions in the early postoperative period. The aim of this study was to understand whether procalcitonin (PCT) values within 48 hours of surgery could be a useful marker of postoperative infection. DESIGN: Retrospective observational study. SETTING: The study was performed in a teaching hospital. PARTICIPANTS: All patients ≤six years of age. INTERVENTIONS: Cardiac surgery on cardiopulmonary bypass from January 1, 2017 to January 1, 2020. MEASUREMENT AND MAIN RESULTS: PCT, white blood cell count, and C-reactive protein values were measured at intensive care unit admission and at 24 and 48 hours after surgery. All positive cultures in the first seven days after surgery were recorded. Out of 177 consecutive patients, 22 (12%) developed infections. PCT at 48 hours after surgery was the only laboratory predictor of infections in the first seven days after surgery (p = 0.02). Receiver operating curve analyses on PCT values at 48 hours identified an optimal cut-off value of 1.85 ng/mL in the overall population. Area under the curve was 0.63, sensitivity 63%, and specificity 69%. CONCLUSIONS: In light of this preliminary result, the clinical relevance and predictive accuracy of PCT are promising in patients with increasing values of PCT but need to be confirmed in a larger sample.

Pediatric Extraskeletal Ewing Sarcoma Originating in the Heart: A Case Report and Review of the Literature.

Extraosseous Ewing sarcoma of primary cardiac origin is an extremely rare variety among pediatric cardiac neoplasms. We report a case of extraosseous Ewing sarcoma of primary cardiac origin in a 9-year-old girl, treated with debulking surgery, adjuvant chemotherapy, and radiotherapy.

Isolated Congenital Coronary Ostial Stenosis in a Young Infant: A Case Report.

Coronary ostial stenosis is a rare congenital cardiac anomaly, frequently associated with hypoplasia of the proximal coronary artery. This condition is potentially life-threatening, as it may present with myocardial ischemia and sudden death. We present a case of left coronary ostial stenosis in a 48-day-old infant symptomatic for sudden cardiac arrest, who successfully underwent surgical angioplasty. Any cardiac arrest in a neonate or young infant should raise suspicion of coronary ostial stenosis/atresia, considering the difficulty in diagnosing this congenital heart defect.

Transposition of the great vessels and intact ventricular septum: is there an age limit for the arterial switch? Personal experience and review of the literature.

OBJECTIVE: Prognosis of the transposition of the great arteries has completely changed since the introduction of the arterial switch. Time limit to perform this intervention is still controversial. The aim of this study is to demonstrate the early and late outcome of primary arterial switch operation beyond the age of months. METHODS: We included all patients with the diagnosis of transposition of the great arteries with intact ventricular septum beyond the age of 8 weeks who underwent primary arterial switch operation. The procedures were performed by the same surgeon, in two different institutes. Patients who had transposition of the great arteries and associated anomalies (except atrial septal defect and persistent arterial duct) were excluded. Ventricular shape, geometry, and mass were not considered during the decision on procedure type. RESULTS: In the study, 11 patients with the diagnosis of simple d-transposition of the great arteries beyond 8 weeks were undergone primary arterial switch operation with a mean age of 90.63 days (60-137 days), and 7 patients had a Rashkind procedure. All patients had squashed left ventricle shape with preserved function. The sternum was left open in 10 patients. Extracorporeal membrane oxygenation support was necessary in 45.45% of cases. The mean mechanical ventilation time was 7.27 days (1-16 days). No mortality was recorded until now. Post-operatory left ventricular function was preserved in 90.9% of the patients. Only one patient had mild myocardial dysfunction at the time of discharge. CONCLUSIONS: Primary arterial switch procedure can still be the best surgical option in patients with the diagnosis of transposition of the great arteries with intact ventricular septum beyond 8 weeks of age, providing that mechanical circulatory support and an expert cardiac intensive care unit service are available.

Aorto-Right Ventricular Tunnel: A Case Series and Literature Review.

Aorto-right ventricular tunnel (ARVT) is a rare cardiac congenital anomaly where an extracardiac channel connects the ascending aorta above the sinutubular junction to the right ventricle. This defect is caused by an abnormal development of the cushions of the aorto-pulmonary outflow tract. A case series and literature review are described. Two cases of ARVT are described. A literature review was conducted, in which 31 cases were reported. In our 2 cases, both ARVTs connected the ascending aorta above the left aortic sinus to the right ventricle (one to the right ventricular outflow tract and one to the right ventricular apex). Both patients underwent successful surgical correction by patch closure of both tunnel orifices, with uneventful postoperative course. Of the 31 ARVT cases described in our review, only 10 patients (32.3%) had an anatomy similar to the 2 cases described. Coronary artery anomalies can be associated, as reported in our 2 patients and in 16 cases (51.6%) in the review. Surgical correction can be achieved by direct closure or, more often, by patch closure of one or both tunnel orifices, depending mostly on coronary anatomy. Two cases of transcatheter device closure were described in literature, in favorable anatomy cases. Careful attention is required during repair to avoid coronary lesions, due to the high incidence of comorbid coronary anomalies.

Antibiotic Management for Delayed Sternal Closure Following Pediatric Cardiac Surgery: A Systematic Review of Recent Literature.

Delayed sternal closure after pediatric cardiac surgery is a management option for the treatment of patients with severely impaired heart function. The optimal antimicrobial treatment strategy for this condition is unknown. The aim of this systematic review was to evaluate the current antibiotic administration attitudes in pediatric cardiac surgery patients needing an open chest in terms of infection with a focus on surgical site infection rate. The authors performed a systematic review and meta-analysis of all articles, which described the antibiotic administration strategy and surgical site infection rate in pediatric patients with an open chest after cardiac surgery. The authors performed a subgroup analysis on "standard" versus "non-standard" (defined as any antimicrobial drugs different from the adult guidelines recommendations) therapy for one-proportion meta-analysis with a random effect model. The authors identified 12 studies published from January 1, 2000 to July 1, 2019 including a total of 2,203 patients requiring an open chest after cardiac surgery, 350 of whom (15.9%) developed infections and 182 (8.3%) developed a surgical site infection. The surgical site infection rate in patients with "non-standard" strategy was higher than in patients with "standard" strategy: 8.8% (140 reported infections/1,582 patients) versus 6.8% (42 reported infections/621 patients), p = 0.001. The "standard" antibiotic management proposed by guidelines for adult cardiac surgery patients could be used an acceptable strategy to treat pediatric patients with an open chest after cardiac surgery.

Hybrid approach for disconnected pulmonary arteries: never lose a pulmonary artery again!

Disconnection of a pulmonary artery needs early surgical treatment in order to support the growth of the vessel. However, owing to the high rate of re-stenosis after traditional surgical reconstruction, we developed a hybrid approach involving the creation of pulmonary artery continuity by using autologous or heterologous tissue supported by stent implantation.

An alternative technique for Fontan completion in patients with widely separated connections of the inferior systemic veins to the atrial mass.

Several surgical approaches for completion of a total cavopulmonary connection have been established for patients with anomalies of systemic venous connection in association with a functionally univentricular heart. We report an alternative technique designed to address the problem of widely separated inferior systemic veins, in which two extracardiac conduits were used. This technique was performed on three patients and yielded excellent short-term results, allowing us to operate on a beating heart with no need for cardioplegic arrest and aortic cross-clamping.

Paediatric cardiac surgery in a peripheral European region: is a joint programme a safe alternative to regionalisation?

BACKGROUND: In 2007, a partnership was initiated between a small-volume paediatric cardiac surgery unit located in Las Palmas de Gran Canaria, Spain, and a large-volume cardiac surgery unit located in Milan, Italy. The main goal of this partnership was to provide surgical treatment to children with CHD in the Canary Islands. METHODS: An operative algorithm for performing surgery in elective, urgent, and emergency cases was adopted by the this joint programme. Demographic and in-hospital variables were collected from the medical records of all the patients who had undergone surgical intervention for CHD from January, 2009 to March, 2013. Data were introduced into the congenital database of the European Congenital Heart Surgeons Association Congenital Database and the database was interrogated. RESULTS: In total, 65 surgical mission trips were performed during the period of this study. The European Congenital Heart Surgeons Association Congenital Database documented 214 total patients with a mean age at operation of 36.45 months, 316 procedures in total with 198 cardiopulmonary bypass cases, 46 non-cardiopulmonary bypass cases, 26 cardiovascular cases without cardiopulmonary bypass, 22 miscellaneous other types of cases, 16 interventional cardiology cases, six thoracic cases, one non-cardiac, non-thoracic procedure on a cardiac patient with cardiac anaesthesia, and one extracorporeal membrane oxygenation case. The 30-day mortality was 6.07% (13 patients). CONCLUSIONS: A joint programme between a small-volume centre and a large-volume centre may represent a valid and reproducible model for safe paediatric cardiac surgery in the context of a peripheral region.

Partial abnormal drainage of superior and inferior caval veins into the left atrium: two case reports.

Abnormal connection of the right superior caval vein to the left atrium is an uncommon systemic vein drainage anomaly, with only a few cases reported among congenital heart disease (CHD), around 20 cases published in the medical literature. The inferior vena cava connection with the left atrium, also very rare, can appear directly or in heterotaxy. Clinical suspicion arises due to the presence of cyanosis in the absence of other specific clinical signs (without other associated CHD). We present the cases of two children with abnormal superior and inferior systemic venous return. The first case is an abnormal connection of right superior vena cava to the left atrium associated with persistent left superior vena cava draining into the right atrium through the coronary sinus. The second case is an interruption of the inferior vena cava with hemiazygos continuation, drained into the left superior vena cava, which drained into the left atrium. The diagnosis was imagistic - echocardiography and angiography. Surgical treatment solutions vary from one case to another, usually following anatomic correction. Hypoxia accompanied by cyanosis must bring into question the pathology of systemic venous drainage anomaly, after other common causes have been excluded. Surgery is indicated in all cases due to the risk associated with the presence of right-to-left shunt.

Retuning mortality risk prediction in paediatric cardiac surgery: the additional role of early postoperative metabolic and respiratory profile.

OBJECTIVES: The existing risk stratification scores for paediatric patients undergoing cardiac surgery include the Aristotle Basic Complexity (ABC) Score, the Risk Adjustment in Congenital Heart Surgery-1 (RACHS-1) Score and the Aristotle Comprehensive Complexity (ACC) Score. They are all based on the nature of the surgical operation (ABC and RACHS-1 Scores) with possible adjustment for a number of patient conditions (ACC Score). The present study investigates if the early postoperative parameters may be used to improve the preoperative mortality risk prediction. METHODS: A retrospective study on 1392 consecutive patients aged ≤12 years old, undergoing cardiac surgery with cardiopulmonary bypass and without a residual right-to-left shunt was conducted. The ABC Score and metabolic and respiratory postoperative parameters at arrival in the intensive care unit were tested for association and discriminative power for operative mortality. RESULTS: The ABC yielded a c-statistic of 0.746. Additional independent predictors of operative mortality were postoperative hypoxia [Formula: see text] and arterial blood lactates. In a multivariable model including the ABC Score, postoperative hypoxia and arterial blood lactates remained independently associated with operative mortality. A modified ABC Score was created, consisting of the ABC Score plus 1.5 points in case of postoperative hypoxia plus 1 point per each 1 mmol/l of arterial blood lactates. The new model was significantly (P = 0.043) more discriminative than the ABC Score, with a c-statistic of 0.803. CONCLUSIONS: Early postoperative respiratory and metabolic parameters increased the accuracy and discrimination of the ABC Score. An external validation is needed to confirm our results.

[A late diagnosis of left atrial myxoma: how to gain time?] / Una diagnosi tardiva di mixoma atriale sinistro. Come guadagnare tempo?

A 56-year-old man presented to the Outpatient Cardiology Unit for dyspnea that had been lasting 6 months and an occasional episode of cold perspiration on climbing a flight of stairs. In the suspicion of coronary artery disease, he was prescribed a complete blood panel, an echocardiogram and a treadmill stress test. The echocardiogram, performed as late as 78 days after the first evaluation and only by chance scheduled 2 days before the stress test, enabled a diagnosis of left atrial myxoma for which the patient successfully underwent cardiac surgery. The authors discuss the aspecific and potentially misleading nature of myxoma symptoms and highlight the latency between cardiological evaluation and diagnostic echocardiography.

The care for adults with congenital heart disease: organization and function of a grown-up congenital heart disease unit.

Thanks to the improvement of surgical and interventional cardiac procedures, the majority of children with congenital heart defects (CHD) can now be expected to reach adulthood. The number of adults with congenital heart disease (ACHD) will inevitably increase, and recent data affirm that in Europe, we are actually faced with an estimated patient population of 2.3 million. These patients, particularly the adults with moderate and highly complex CHD, can be very difficult to manage, and should be treated in few experienced and specialized 'grown-up congenital heart disease' (GUCH) units, concentrating resources, patients, funding, and professional experiences. On 2000, we created a GUCH unit in our hospital that rapidly became a referral centre in Italy for number and complexity of patients treated. The present study is a presentation of how is organized our GUCH unit and a brief overview of our more recent experience with ACHD patients.

International cooperation in healthcare: model of IRCCS Policlinico San Donato and Bambini Cardiopatici nel Mondo Association for congenital heart diseases.

Cooperation activity in training programmes promoted by IRCCS Policlinico San Donato and Bambini Cardiopatici nel Mondo Association is a model of cooperation between people as an alternative intervention in promoting the right to healthcare, especially offering programmes of training and medical care on cardiovascular and congenital heart disease. This new strategy, implemented in several developing countries, has absolutely contributed to the improvement of the medical services concerning the diagnostic and surgical approach in the treatment of paediatric and adult cardiovascular disease. To strengthen this kind of activity, both IRCCS Policlinico San Donato and Bambini Cardiopatici nel Mondo have introduced a global perspective aiming at the realization of surgical missions 'in situ', building new cardiac surgery units in collaboration with the local partners, which are mainly university hospitals. They, furthermore, support financially the scholarship and accommodation in favour of medical and paramedical staff.

Fontan operation for patients with complex anatomy: the intra-atrial conduit technique.

The extracardiac conduit type of total cavopulmonary connection (TCPC) is the most common variation of the modified Fontan operation in current use. For patients with some forms of complex anatomy (eg, dextrocardia in situs solitus or asplenia syndrome), we have adopted a different technique: interposition of an intra-atrial conduit between the inferior vena cava (IVC) and the superior vena cava-right pulmonary artery (SVC-RPA) connection. We report our experience with six patients.

Cor triatriatum dexter: unusual features in utero and after birth.

Cor triatriatum dexter is a rare congenital anomaly, caused by the persistence of the right valve of the sinus venosus, which divides the right atrium into two chambers and its diagnosis is rather difficult, due to the features often mimicking Ebstein's anomaly. We describe a case followed from the foetal age for a relative right ventricular hypoplasia and suspicious pulmonary stenosis and diagnosed after birth to have cor triatriatum dexter with an obstructing membrane in the right atrium, needing surgical correction, done successfully.