Staged approach for correction of anomalous venous return to the azygos vein.

Partial anomalous pulmonary venous return into the azygous vein is a rare pathological finding. We describe the case of a 28-year-old girl who had a successful staged approach to treat this rare congenital heart disease. To avoid potential connection of a systemic venous return to the left atrium, the proximal part of the azygous vein was occluded with a percutaneous approach, then the azygous vein flow was redirected into the left atrium with a surgical procedure.

Everolimus-induced near-resolution of giant cardiac rhabdomyomas and large renal angiomyolipoma in a newborn with tuberous sclerosis complex.

We report a case of a newborn, affected by tuberous sclerosis complex, with a prenatally diagnosed giant cardiac rhabdomyoma associated with a large renal angiomyolipoma presenting as a duct-depending lesion not treatable by surgery. After receiving everolimus, a mammalian target of rapamycin inhibitor, we observed a rapid, significant, and durable reduction of both lesions without remarkable side effects.

Anterior and posterior leaflets augmentation to treat tricuspid valve regurgitation.

In congenital non-Ebstein anomalies of the tricuspid valve, the septal leaflet is often involved and tethered. We describe a standardized approach to address septal leaflet tethering by concomitant augmentation of the anterior and posterior leaflets.

The concept of cone creation to treat isolated tricuspid valve dysplasia and the case of a double-orifice tricuspid valve.

Objectives: Isolated tricuspid valve dysplasia is a rare disease characterized by a wide spectrum of possible anomalies. We describe the use of the Cone concept to treat a patient with a double-orifice tricuspid valve with massive regurgitation and severe deficit of coaptation. Methods: Three adult patients with congenital non-Ebstein tricuspid valve anomaly characterized by severe coaptation deficiency underwent tricuspid valve repair applying the Cone technique. In particular, we describe the case of a symptomatic 21-year-old woman with a double-orifice tricuspid valve, with massive regurgitation and severe right ventricular dilatation. The tricuspid valve was transformed from a double-orifice valve into a single-orifice valve. The most superior orifice was opened, and the tissue surrounding the orifice was used to extend the leaflet of the inferior orifice. A Cone was created, and a ring annuloplasty was used to stabilize the result. Results: The patient was discharged home after 7 days with trivial residual tricuspid regurgitation and no significant antegrade gradient. The final coaptation height was 2.8 cm. The cardiothoracic ratio decreased from 0.77 to 0.59 after 2 months, and symptoms promptly improved. Conclusions: Over the past 2 years, we have applied the Cone creation concept to patients with a severely dysplastic tricuspid valve with excellent early results. One patient had a double-orifice tricuspid valve, and a Cone repair concept was adopted anyway. One orifice was sacrificed, and surrounding tissue was used to augment the leaflets of the other orifice. A Cone was created to improve central coaptation with a good initial result.

Bentall Endocarditis by C. Lusitaniae After COVID-19: The Finger Covers The Moon.

We report a case of endocarditis months after a Bentall procedure. This was caused by Candida Lusitaniae, in an immunocompetent patient with a recent SARS-CoV-2 infection. The patient underwent a new Bentall procedure. SARS-CoV-2 has been associated with co-infection by Candida species since the beginning of the pandemic, nevertheless, Candida Lusitaniae remains a very uncommon causative agent of prosthetic endocarditis. We suggest a possible role of the SARS-CoV-2, which may have delayed the diagnosis of endocarditis and the appropriate therapy.

Mitral valve damage after an aortic balloon valvuloplasty in an infant.

The case of a 2-year-old infant admitted to our Institution with a diagnosis of severe aortic valve stenosis is presented. After a balloon valvuloplasty with no results in terms of gradient reduction, an echocardiogram showed a moderate mitral regurgitation. The patient underwent surgical repair of both the aortic and mitral valves. Inspection of the mitral valve showed a 5-mm hole in the posterior leaflet at the P2 scallop. Probably, the dilation tore a secondary cord, pulling away a piece of the leaflet. A quadrangular resection was performed with good results.

Brachial arteriovenous fistula in patients with cavopulmonary connection and poor ventricular function: a bridge to Fontan operation.

OBJECTIVE: Augmentation of pulmonary blood flow is the only surgical treatment to reduce symptoms of cyanosis in patients with cavopulmonary connection unsuitable for Fontan operation. Brachial arteriovenous fistula is a good option to increase pulmonary blood flow. In this report, we analyze its possible consequences on ventricular function. METHODS: Six patients considered unsuitable for a Fontan completion because of poor ventricular function underwent brachial arteriovenous fistula. The fistula was performed with the interposition of a 4-mm gore-tex conduit between the brachial vein and the brachial artery. RESULTS: The mean oxygen saturation increased from 76.8 ± 1.9% to 84.1 ± 1.4% (p < 0.01); hemoglobin and hematocrit decreased from 21.13 ± 0.7 g/dL to 18.12 ± 0.5 g/dL (p < 0.01) and from 63.3 ± 2.7% to 54.2 ± 0.7% (p < 0.01), respectively. Mean ejection fraction and shortening fraction increased from 38.6 ± 1.5% to 49.5 ± 2.3% (p < 0.01) and from 23.3 ± 1.8% to 28.8 ± 1.3% (p < 0.01), respectively. A Fontan completion and takedown of the fistula was then accomplished, within six years, with no mortality or major complications. At a mean follow-up of 15 months, all six patients are clinically in good condition. CONCLUSION: In patients with a cavopulmonary connection and poor ventricular function, the brachial arteriovenous fistula may play a role in increasing oxygen saturation, optimizing ventricular preload, and reducing blood viscosity and pulmonary arteriovenous malformations. This approach might represent an intermediate step to rescue patients previously considered unsuitable or at high risk for Fontan operation.

Myocardial oxygen consumption during histidine-tryptophan-ketoglutarate cardioplegia in young human hearts.

OBJECTIVES: Energy demand and supply need to be balanced to preserve myocardial function during paediatric cardiac surgery. After a latent aerobic period, cardiac cells try to maintain energy production by anaerobic metabolism and by extracting oxygen from the given cardioplegic solution. Myocardial oxygen consumption (MVO2) changes gradually during the administration of cardioplegia. METHODS: MVO2 was measured during cardioplegic perfusion in patients younger than 6 months of age (group N: neonates; group I: infants), with a body weight less than 10 kg. Histidine-tryptophan-ketoglutarate crystalloid solution was used for myocardial protection and was administered during a 5-min interval. To measure pO2 values during cardioplegic arrest, a sample of the cardioplegic fluid was taken from the inflow line before infusion. Three fluid samples were taken from the coronary venous effluent 1, 3 and 5 min after the onset of cardioplegia administration. MVO2 was calculated using the Fick principle. RESULTS: The mean age of group N was 0.2 ± 0.09 versus 4.5 ± 1.1 months in group I. The mean weight was 3.1 ± 0.2 versus 5.7 ± 1.6 kg, respectively. MVO2 decreased similarly in both groups (min 1: 0.16 ± 0.07 vs 0.36 ± 0.1 ml/min; min 3: 0.08 ± 0.04 vs 0.17 ± 0.09 ml/min; min 5: 0.05 ± 0.04 vs 0.07 ± 0.05 ml/min). CONCLUSIONS: We studied MVO2 alterations after aortic cross-clamping and during delivery of cardioplegia in neonates and infants undergoing cardiac surgery. Extended cardioplegic perfusion significantly reduces energy turnover in hearts because the balance procedures are both volume- and above all time-dependent. A reduction in MVO2 indicates the necessity of a prolonged cardioplegic perfusion time to achieve optimized myocardial protection.

[Modified Warden procedure for correction of right superior vena cava drainage in the left atrium]. / Warden modificata per la correzione del drenaggio anomalo della vena cava superiore in atrio sinistro.

A newborn with situs solitus, normally related great arteries and intact atrial septum, underwent surgical repair at our institution for anomalous drainage of the right superior vena cava in the left atrium at the level of the right superior pulmonary veins. This rare cyanotic congenital cardiac malformation is herein described with special regard to its anatomical and diagnostic features. A novel surgical approach for achieving correction is also described.

A Misdiagnosed Case of Double Outlet Right Atrium Associated With Hypoplastic Right Ventricle.

Double outlet right atrium (DORA) is a rare congenital heart disease in which the right atrium opens into both ventricles. The reduced leftward motion of the interventricular septum causes a malalignment between the atrial and the ventricular septum at the cardiac crux, which is the pathognomonic feature of this heart defect. We describe a case of significant exertional desaturation in an adult patient who was diagnosed with DORA, restrictive right ventricle, and anomalous tricuspid valve. Subsequently, the patient underwent one-and-a-half ventricular palliation.

Aortopulmonary Collateral Artery from the Proximal Ascending Aorta: A Rare Anatomical Finding.

Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries is a rare congenital heart lesion in which pulmonary blood supply may arise from different segments of the aorta. We report an unusual case of a newborn with a major collateral artery originating from the proximal ascending aorta. Successful reparative surgery was undertaken.

[Anomalous origin of the right coronary artery from the pulmonary artery or coronary artery fistula: when the diagnosis is uncertain]. / Quando la diagnosi può essere incerta: origine anomala dell'arteria coronaria destra dall'arteria polmonare o fistola coronarica?

The anomalous origin of the right coronary artery from the pulmonary artery is a rare congenital coronary anomaly with a reported incidence of approximately 0.002%. Usually, the diagnosis is made by echocardiography leaving computed tomography or angiography only to diagnostic completion in doubtful cases or for interventional procedures.Herein we report a doubtful case of a patient with a diagnosis of coronary fistula between the right coronary and the pulmonary artery that proved to be an anomalous origin of the coronary artery from the pulmonary artery. The patient underwent corrective surgery with translocation of the coronary artery on the aorta.

Evolution of a rare ECG pattern in an aggressive case of neonatal tuberous sclerosis complex.

INTRODUCTION: Rhabdomyomas are the most frequent cardiac tumors in children. Furthermore, they are often associated to tuberous sclerosis complex, an autosomal dominant neurocutaneous disorder characterized by tumor-like malformations that involve many organ systems. PRESENTATION OF THE CASE: We describe a rare ECG pattern in a severe case of neonatal tuberous sclerosis complex. DISCUSSION: In the presence of significant rhabdomyomatosis related to tuberous sclerosis, multiple clusters of rhabdomyoma-like cells can infiltrate the myocardium, with increased fibrosis areas. CONCLUSION: Considering the fact that rhabdomyomas often show spontaneous regression, close follow-up is sufficient in hemodynamically stable cases. Destruction of the conduction system, with arrhythmias as consequence, can be the presenting feature of diffuse rhabdomyomatosis.

Outcome of long-term complications after permanent metallic left bronchial stenting in children.

OBJECTIVES: We describe the way we treated 7 children with critical long-term complications after metallic balloon-expandable stenting in the left mainstem bronchus. METHODS: Endoscopic follow-up included a first bronchoscopy 3 weeks after stenting, then monthly for 3 months, every 4-6 months up to 1 year and at scheduled times to calibrate stent diameter up to final calibration. When major complications occurred, patients underwent chest computed tomographic angiography. RESULTS: In 1 of the 7 children (median age 2.8 years), metallic left bronchial stenting served as a bridge to surgery. After a median 4-year follow-up, all 7 children experienced recurrent stent ovalizations with stent breakage in 3 and erosion in 1. In 4 children, computed tomographic angiography showed abundant peribronchial fibrous tissue, in 2 left mediastinal rotation and in 1 displacement along the left bronchus after pulmonary re-expansion as the cause of stent-related complication. Of the 7 children, 6 underwent surgery (5 posterior aortopexy and 1 section of the ligamentum arteriosus) and 3 required nitinol stents placement within the metallic ones. One patient completed the follow-up, and 1 patient was lost to follow-up. All 5 remaining children still have permanent bronchial stents in place, patent and re-epithelialized after a median 10.5-year follow-up. There were no deaths. CONCLUSIONS: Satisfactory anatomical relationships when children have stents placed in the left mainstem bronchus alone do not guarantee the final success. Several mechanisms intervene to cause critical stent-related complications in children during growth. Permanent metallic stents should be used carefully, and only in selected patients.

Obstructive neonatal atrial myxoma.

INTRODUCTION: Cardiac myxoma is a benign neoplasm representing the most common primary cardiac tumor in adults, however it is unusual in neonates. It is represented by an endocardial mass that occupies the cardiac chamber. Although the majority of myxomas are attached to the fossa ovalis of the interatrial septum, they also attach to the walls of the cardiac chambers and to valve leaflets surfaces. Approximately 75% of myxomas are found in the left atrium, 20% are located in the right atrium, and rarely in the ventricles. PRESENTATION OF CASE: We describe a rare case of neonatal cardiac myxoma arising from interatrial septum, causing significant mechanical obstruction to blood flow through tricuspid valve, in an otherwise normal newborn. The patient underwent successful excision of the myxoma with an uneventful recovery [1]. DISCUSSION: Cardiac tumors are rare in children, thus an understanding of the common types of benign and malignant paediatric cardiac tumors and their imaging features, is important because the epidemiology and tumor types differ from those encountered in adults. CONCLUSION: Large neonatal myxoma is exceptionally rare and even more infrequent is the surgical excision in the first day of life.

Reversible Closure of the Ductus Arteriosus.

There are cases in which ligation of the ductus arteriosus is part of a palliative or reparative procedure where the opportunity to reopen the ductus in the early period after surgery could be beneficial. This applies, particularly, to patients with borderline left ventricle in whom a biventricular repair is performed or in small babies in whom a modified Blalock-Taussig shunt is performed, particularly when a very small polytetrafluoroethylene graft is used. Conventional ductal ligation is generally irreversible. We describe a technique of ductal closure that allows easy reopening of the duct in the early postoperative period.

Paediatric Nonfunctioning Adrenocortical Carcinoma with Extension up to Right-Side Heart: Cardiac Surgery Approach.

Adrenocortical carcinoma is a rare malignancy. Due to late diagnosis and no adequate effective adjuvant treatment, prognosis remains poor. Only approximately 30% of these malignancies are confined to the adrenal gland when they are diagnosed, as these tumors tend to be found years after their genesis. Cardiac involvement of adrenal carcinoma is very rare. We report a rare case of a 7-year-old female with right adrenal cortical carcinoma, involving the right-side heart.