Single-incision, off-pump repair of a right aortic arch with an aberrant left subclavian artery and diverticulum of Kommerell in an adult.

Repairing a complete vascular ring in adults can be challenging. A right aortic arch with an aberrant retro-oesophageal left subclavian artery and persistent diverticulum of Kommerell represents one of the most common variants seen in adults, and the ring is completed by the left-sided ligamentum arteriosum. Most presentations in adults occur secondary to oesophageal compression, resulting in varying degrees of dysphagia. Owing to the difficulty and challenges associated with exposure in adults, it is not unusual for surgeons to offer a two-incision approach or to stage the procedure. We present a detailed surgical technique for a single-incision repair of a right aortic arch with an aberrant retro-oesophageal left subclavian artery via a left posterolateral thoracotomy.

Bilateral branch pulmonary arterial reconstruction in the Alagille syndrome: The technique and its pitfalls.

This tutorial describes in detail the surgical technique of bilateral branch pulmonary arterial reconstruction in a 10-month-old boy with Alagille syndrome and advanced liver disease. The procedure was performed via a standard median sternotomy and moderate hypothermia and involves bilateral pulmonary branch arterioplasties combined with relief of valvular and supravalvular pulmonary stenosis and subtotal closure of secundum atrial septal defect. The patient presented with systemic/suprasystemic right ventricular pressure.

Extracardiac Fontan completion after the Kawashima procedure with a custom-made bifurcated graft.

The patient is a 5-year-old girl who underwent a previous Kawashima procedure with a left-sided bidirectional cavopulmonary anastomosis as a stage I palliation for her functional single ventricle. Her cardiac defect consisted of an unbalanced, right-dominant complete atrioventricular septal defect and a double outlet right ventricle. She also had heterotaxy syndrome with left isomerism, polysplenia, and an interrupted inferior vena cava with azygous continuation to a right-sided superior vena cava. Her native main pulmonary artery was left in continuity with her branch pulmonary arteries. She developed sinus node dysfunction, dilated ascending aorta, and progressive cyanosis. We proceeded with the completion Fontan using a bifurcated graft from both hepatic veins to the pulmonary arterial confluence, replacement of her dilated ascending aorta, disconnection of her native main pulmonary artery with excision of the pulmonary valve cusps, and placement of a dual-chamber epicardial pacemaker.

Modified Barbero-Marcial repair for type I truncus arteriosus in a 2.7-kg neonate.

Truncus arteriosus is a rare cardiac anomaly, accounting for less than 4% of all congenital lesions. It is the result of failed aorticopulmonary septation during the fifth week of gestation leading to a single arterial trunk overriding the interventricular septum, a single semilunar valve, and typically a large conotruncal ventricular septal defect. Several classifications exist, and it typically requires surgical repair in the neonatal period. We present a 5-day old female neonate who was diagnosed postnatally with type I truncus arteriosus in which the pulmonary arteries arose from a discrete pulmonary trunk that originated from the posterolateral aspect of the common arterial trunk. A successful repair was undertaken using a variant of the Barbero-Marcial technique.

Left heart bypass for repair of aortic coarctation in children: Technical tips and pitfalls.

Spinal cord injury secondary to coarctation repair in children is a serious, though relatively uncommon, complication. Several measures have been proposed to minimize this risk, which seems to affect more older children than neonates and those with inadequate collateral circulation. Left heart bypass has been proposed as a protective strategy.

Left Thoracotomy Beating Heart Repair of Mitral Periprosthetic Regurgitation.

Left thoracotomy is a recognized, albeit not a commonly used, approach for mitral valve exposure. This approach represents a good alternative to repeat median sternotomy and repeat right thoracotomy, especially in cases of hostile mediastinum. We present a 72-year-old woman who underwent a beating heart left thoracotomy repair of recurrent severe mitral periprosthetic regurgitation in the presence of a porcelain aorta.

Intraoperative fluorescence with indocyanine green in congenital cardiac surgery: Potential applications of a novel technology.

BACKGROUND: Achieving successful repair of congenital heart defects requires attention to detail. Careful evaluation of anastomotic site patency, reimplanted coronary arteries, location of epicardial coronaries, and myocardial perfusion may be necessary to ensure safe conduct of the operation and achieve excellent outcomes. METHODS: Intraoperative fluorescence angiography (IOFA) was performed using indocyanine green in patients undergoing the following procedures: coronary artery reimplantation, coronary artery unroofing, evaluation of intraoperative coronary anatomy, reoperation requiring ventriculotomy, coarctation/interrupted aortic arch repair, systemic-to-pulmonary artery shunt, Norwood procedure, thoracic duct localization, and vascular rings. RESULTS: The technique was feasible in all patients. No mortality or adverse events related to the dye were encountered. Adequate intraoperative imaging was obtained in 15 patients (20 images), which correlated well with postoperative studies. The imaging quality was superior for assessing shunt patency, myocardial perfusion, and anastomotic patency and determining the location of epicardial coronary arteries but was less so for assessing branch pulmonary arteries. CONCLUSIONS: IOFA is a useful and safe technique that can provide a quick on-table assessment of a variety of congenital surgical procedures. This may help answer questions that can minimize postoperative interventions and help ensure a smooth perioperative course and excellent outcomes.

Off-pump extracardiac Fontan completion: Surgical technique and pitfalls.

Completion of the extracardiac Fontan procedure is the final palliative stage for treating a functional single ventricle. It has been associated with a smaller incidence of atrial arrhythmias and more laminar flow in the Fontan pathway. We present our technique for the off-pump extracardiac Fontan procedure.

Pulmonary valve replacement via left anterior minithoracotomy: Lessons learned and early experience.

OBJECTIVE: Median sternotomy has been the standard for pulmonary valve replacement (PVR) in patients with free pulmonary regurgitation (PR) and right ventricular enlargement. With the introduction of transcatheter therapy, the search for an alternate to sternotomy is mandated. We present our early experience with a limited anterior left thoracotomy approach. METHODS: We used a left anterior mini-thoracotomy in six male patients (15 ± 1.94 years of age) who developed progressive right ventricular enlargement due to chronic PR. RESULTS: Primary diagnoses were tetralogy of Fallot in five patients and pulmonary atresia with an intact septum in another. Four patients had previous median sternotomy with transannular patch repair. The mean right ventricular end-diastolic volume index was 189 ± 27.13 ml/m2 . The procedure was feasible in all patients. All patients had satisfactory adult size pulmonary bioprosthesis (25 or 27 mm valve), with a mean peak gradient of 18 ± 2.40 mmHg across the prosthesis at discharge. All patients were extubated intraoperatively at the end of the procedure and required no intraoperative transfusions. There were no early or late mortalities. Early morbidities included left hemidiaphragm paralysis in one patient, and re-sternotomy for prosthetic valve endocarditis in one. One patient required late reoperation for a common femoral artery pseudoaneurysm. CONCLUSIONS: Minimally invasive access for PVR is feasible in both primary and repeat settings, through a limited anterior left minithoracotomy in the absence of intracardiac shunts and the need for other concomitant cardiac procedures. Longer-term studies with a larger number of patients are needed to compare the efficacy of this approach to standard sternotomy.

Outcomes of Surgical Repair of Vascular Rings and Slings in Children: A Word for the Asymptomatic.

Vascular rings (VRs) are rare aortic arch anomalies that may present with a wide variety of symptoms related to esophageal and/or airway compression. We reviewed our surgical experience in both symptomatic and asymptomatic children. All children (n = 58) who underwent surgical repair of VRs or slings (mean age 27.4 ± 45.60 months; 36 males [62%]) between March 2000 and April 2020 were included. The most common anatomic variant was a right aortic arch (RAA) with aberrant left subclavian artery (ALSCA) (n = 29; 50%). Kommerell's diverticulum was present in 23 of these patients (79%). The second most common variant was a double aortic arch (n = 22; 38%), followed by pulmonary artery sling (n = 4; 6%), RAA with mirror image branching and left ligamentum arteriosum (n = 3; 5.2%), and left aortic arch (LAA) with aberrant right subclavian artery (n = 1; 1.7%). One patient had a double ring with pulmonary artery sling and RAA with ALSCA. Symptoms were present in 42 patients (72%). Left lateral thoracotomy was the approach in 50 patients (86%), while sternotomy was used in 8 (14%). Symptomatic improvement occurred in the majority of symptomatic patients (93%). There was one perioperative mortality (1.7%) in the symptomatic group which was non-VR related. Morbidities included recurrent laryngeal nerve injury in three patients (5.2%) and transient chylothorax in two (3.4%). Persistence/recurrence of symptoms resulted in one early and one late reoperation. The mean follow-up was 3 ± 5 years. In the current era, VR repair in children including asymptomatic ones can be performed with excellent results. We recommend complete repair of RAA with aberrant LSCA by resection of Kommerell's diverticulum and translocation of the ALSCA to avoid recurrence.

Berlin Excor Cannulation of Left Atrial Appendage in Left Ventricular Restrictive Physiology: A Novel Bailout Strategy.

Ventricular assist device (VAD) management continues to be a challenge in the presence of restrictive physiology. Left atrial (LA) decompression is not satisfactory even with good function and position of the left ventricular cannula. We describe an alternate approach with LA cannulation via the left atrial appendage (LAA) as a rescue strategy in a patient who had restrictive physiology, in our case was secondary to viral myocarditis acute systolic heart failure with subsequent insidious diffuse endomyocardial fibrosis and superimposed massive calcification, causing inadequate emptying of the left ventricle despite optimal VAD apical cannula position.

Combined Manouguian-Guiraudon Approach for Double Valve Replacement: The Commando Revisited.

Double annular enlargement is a valuable technique for the cardiac surgeon, and it should be in his or her armamentarium when dealing with challenging aortic and mitral valve cases. The technique requires reconstruction of the intervalvular fibrosa. It redesigns the inflow and outflow of the left ventricle in a way that serves both annular enlargement and satisfactory valve replacement with resulting low gradients. Here, we describe our preferred operative steps.

Orthotopic Heart Transplantation in a Child with Single Ventricle after Pneumonectomy.

We report a 6-year-old with single ventricle physiology secondary to tricuspid atresia who had cardiorespiratory failure who was not a candidate for further single ventricle palliation. The patient underwent planned staged left pneumonectomy for recurrent pneumonias secondary to bronchomalacia followed by orthotopic heart transplantation. This aggressive approach improved the patient candidacy for heart transplantation by removing the source of recurrent infection and respiratory failure (left lung).

Unroofing of Myocardial Bridging After Septal Myectomy in a Child With Noonan Syndrome.

Myocardial bridging is a controversial topic that remained with no well-defined management protocol. We present a ten-year-old child with Noonan syndrome and a myocardial bridge.

Off-Pump "Clamp and Sew" Extracardiac Fontan With Inverted Bifurcated Graft for Dextrocardia With Heterotaxy Syndrome.

Avoiding cardiopulmonary bypass during palliation of single ventricle has the advantages of minimizing transfusions, pulmonary vascular resistance, and avoiding the inflammatory response from cardiopulmonary bypass. It is however not always straightforward, and the technique may be faced with challenges.

Anomalous Aortic Origin of the Right Pulmonary Artery From the Ascending Aorta With Spontaneous Dissection and Thrombosis.

Anomalous aortic origin of the pulmonary artery is rare. It can affect either of the main branches and can be an important cause of neonatal respiratory distress. Early diagnosis and surgical repair is associated with improved survival and long-term outcomes.

Transaortic extended left ventricular septal myectomy in an adult with hypertrophic obstructive cardiomyopathy.

In symptomatic patients with an obstructive variant of hypertrophic cardiomyopathy and no response to maximal medical therapy, we recommend a septal myectomy. It is considered the gold standard for treatment of the basal variant of hypertrophic cardiomyopathy. It has several advantages over alcohol septal ablation, such as the immediate relief of the obstruction and the ability to reduce the septal thickness significantly, to eliminate the potential for midventricular obstruction, and to rule out any other etiologies of left ventricular outflow tract obstruction, which include the presence of abnormalities in the mitral valve subvalvular apparatus such as the presence of anomalous chords, which occured in the current case, and anomalous papillary muscles. In experienced hands, the technique is safe and is associated with excellent outcomes with improved quality of life and potential for survival benefit.