Right cervical aortic arch associated with anomaly of epiaortic vessels and haemodynamically significant obstruction between ascending and descending aorta. Extra-anatomic bypass graft between ascending and descending aorta and long-term follow-up.

Right cervical aortic arch is a rare malformation in which a right-sided aortic arch is unusually long, forms a loop, descends, and crosses behind the oesophagus to continue as a left descending aorta. We present a case of right cervical aortic arch associated with anomaly of epiaortic vessels and aortic obstruction, surgically treated with extra-anatomic bypass graft between ascending and descending aorta.

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.

Congenital left ventricular aneurysm of interventricular septum: prenatal diagnosis and long-term management.

Congenital left ventricular aneurysm is a rare anomaly consisting of an extroflection from left ventricular walls with a wide connection. Most left ventricular aneurysms are asymptomatic and diagnosed as incidental finding, but can be associated with complications during follow-up. We present a case of congenital left ventricular aneurysm at the level of the interventricular septum with secondary remodelling of the left ventricle.

Cardiac surgery in adults with high-surgical complexity CHD: results of a network collaborative programme.

BACKGROUND: Adults with CHD often exhibit complex cardiac abnormalities, whose management requires specific clinical and surgical expertise. To enable easier access of these patients to highly specialised care, we implemented a collaborative programme that incorporates medical and surgical specialists belonging to both paediatric and adult cardiovascular institutions. OBJECTIVES: The objective of this study was to review the experience gained and to analyse the surgical outcome of major cardiac surgery. METHODS: We retrospectively reviewed all consecutive patients admitted for major cardiac surgery using our network between January, 2010 and December, 2013. Analysis of surgical outcome was performed in patients selected for major cardiac surgery with cardiopulmonary bypass. Early and late outcomes were evaluated. RESULTS: Out of a total of 433 inward patients, 86 were selected for surgery. The median age was 25.5 years, -64 patients (74.4%) had previously undergone heart surgery, and -55 patients (64%) had been subjected to at least one sternotomy. Abnormalities of the left ventricular and right ventricular outflow tract were the most frequent (37.2% and 30.2%, respectively), and despite high-surgical complexity only one death occurred (in-hospital mortality 1.1%). On a median follow-up time of 4 years no deaths and no heart-failure events have occurred; one patient underwent further cardiac surgery programmed at the time of discharge. CONCLUSIONS: Low mortality and morbidity rates can be obtained in high-surgical complexity adults with CHD populations when paediatric and adult cardiac specialists operate in the same multidisciplinary environment.

Renal dysfunction in cardiovascular diseases and its consequences.

It is well known that the heart and kidney and their synergy is essential for hemodynamic homeostasis. Since the early XIX century it has been recognized that cardiovascular and renal diseases frequently coexist. In the nephrological field, while it is well accepted that renal diseases favor the occurrence of cardiovascular diseases, it is not always realized that cardiovascular diseases induce or aggravate renal dysfunctions, in this way further deteriorating cardiac function and creating a vicious circle. In the same clinical field, the role of venous congestion in the pathogenesis of renal dysfunction is at times overlooked. This review carefully quantifies the prevalence of chronic and acute kidney abnormalities in cardiovascular diseases, mainly heart failure, regardless of ejection fraction, and the consequences of renal abnormalities on both organs, making cardiovascular diseases a major risk factor for kidney diseases. In addition, with regard to pathophysiological aspects, we attempt to substantiate the major role of fluid overload and venous congestion, including renal venous hypertension, in the pathogenesis of acute and chronic renal dysfunction occurring in heart failure. Furthermore, we describe therapeutic principles to counteract the major pathophysiological abnormalities in heart failure complicated by renal dysfunction. Finally, we underline that the mild transient worsening of renal function after decongestive therapy is not usually associated with adverse prognosis. Accordingly, the coexistence of cardiovascular and renal diseases inevitably means mediating between preserving renal function and improving cardiac activity to reach a better outcome.

[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.

Italian survey on cardiac surgery for adults with congenital heart disease: which surgery, where and by whom?

OBJECTIVES: The population of ageing adults with congenital heart disease (ACHD) is increasing; surgery in these patients presents major difficulties in management. A great debate has developed about whether these patients should be cared for at an adult or paediatric hospital and by an acquired or congenital cardiac surgeon. We analysed data of the surgical treatment of ACHD from the Italian cardiac surgery centres in 2016, focusing on the type of surgery performed, where these patients were operated on and by whom. METHODS: Ninety-two Italian cardiac surgery centres were contacted and 70 centres participated in this study. We collected data on the types of cardiac operations performed in congenital heart defect patients older than 18 years. In 2016, a total of 913 patients with ACHD were operated on: 440 by congenital cardiac surgeons (group I) in centres with paediatric and adult cardiac surgery units, and 473 by adult cardiac surgeons (group II) in centres with exclusively adult cardiac surgery units. RESULTS: Pathologies of the right ventricular outflow tract were the most frequent diseases treated in group I and pathologies of the left ventricular outflow tract in group II. Group I included more complex and heterogeneous cases than group II. Surgery for ACHD represented 12% of the activity of congenital cardiac surgeons and only 1% of the activity of adult cardiac surgeons. CONCLUSIONS: In Italy, ACHD patients are operated on both by congenital and adult cardiac surgeons. Congenital cardiac surgeons working in centres with both paediatric and adult cardiac surgery are more involved with ACHD patients and with more complex cases.

Primary repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a useful approach.

BACKGROUND: The ultimate goal of surgical therapy for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is to create unobstructed and separate in series pulmonary and systemic circuits. Our preference has been a 1-stage complete unifocalization technique, avoiding collateral anastomosis with either the native pulmonary arteries or other aortopulmonary collateral vessels. METHODS AND RESULTS: Since 1998, 5 patients (median age 29.6 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone surgical correction, consisting of (1) exclusion of a descending thoracic aortic segment from which all major aortopulmonary collateral arteries originate, and (2) connection of this aortic segment to the native pulmonary artery using an interposition polytetrafluoroethylene conduit. The ventricular septal defect was closed in all patients, and the right ventricle was connected to the unifocalized pulmonary artery with a valved conduit. All patients survived the operation. Two patients required reexploration for postoperative bleeding. One patient remained on mechanical ventilation for 17 days due to a pulmonary infection. During follow-up (12-21 months), no patient required additional interventions. The postoperative right ventricular/left ventricular pressure ratio was 0.55 median. No significant stenosis within the reconstructed pulmonary circuit was identified. All patients remain free of symptoms, requiring no medications. CONCLUSION: Intracardiac repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries can be accomplished by a midline 1-stage repair including complete unifocalization of all pulmonary blood supply without individual collateral anastomosis in selected patients. This approach offers a convenient and satisfactory surgical option.

Double-outlet right ventricle in a neonate with Cantrell's syndrome.

We report a case of the association of Cantrell's syndrome with double-outlet right ventricle in a neonate.

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.

Implantation of a Berlin Heart as single ventricle by-pass on Fontan circulation in univentricular heart failure.

The clinical management of ventricular failure after the Fontan operation presents a formidable challenge to surgeons. We report our experience with successful implantation of a Berlin Heart EXCOR ventricular assist device as a bridge to transplantation in a child with Fontan circulation.