Anomalous left coronary from the pulmonary artery-clinical profile,operative techniques and patient outcomes

Background Anomalous left coronary artery arising from the pulmonary artery is a rare congenital heart disease. Mortality is in excess of 90% if left untreated during the first year of life. Surgical management has evolved from coronary ligation to anatomical repair by coronary button translocation to the aorta. We report our recent experience with surgical correction of this rare condition. Methods We retrospectively studied the case records of 14 consecutive patients operated in our institute between Jan 1998 to Aug 2004. The mean age was 65 months ±114 (Range 1 to 420). The male to female ratio was 2.5:1. Coronary ligation ws performed in two patients, coronary artery bypass grafting with saphenous vein graft was done in three, the last nine patients underwent coronary button transfer, one patient required concomitant mitral valve replacement. Clinical profile, surgical techniques and operative outcomes as well as long-term results were analyzed. Results Hospital mortality was 28.5% (4/14), Follow up was 100% complete with no late mortality, ranging from 1 months to five years. Ischaemic mitral regurgitation progressed in only one patient, remained the same in one and improved in all the others. All patients except one are in functional class I at last follow up. Conclusion In conclusion, infants presenting with a diagnosis of anomalous left coronary artery arising from pulmonary artery with congestive cardiac failure are a difficult subgroup of patients. Coronary button translocation has emerged as the single most effective surgical modality and is associated with good long-term results.     

Isolated single coronary artery from nonadjacent sinus of Valsalva

We present the computed tomography angiogram images of a 43-year-old lady with an isolated single coronary artery arising from the nonadjacent sinus of aortic root.     

Coronary artery bypass grafting for coronary artery anomalies in infants and young children

Open in a separate window OBJECTIVESCoronary artery bypass grafting (CABG) has been reported for coronary artery diseases in patients with Kawasaki disease and coronary artery complications after arterial switch operations for transposition of the great arteries. However, only a few studies have explored this modality for congenital coronary artery anomalies. As congenital coronary artery anomalies, particularly left coronary artery atresia and stenosis, are one of the reasons for sudden death, coronary revascularization is often required in infants and young children. Therefore, we aimed to investigate the outcome of CABG for such anomalies in infants and young children.METHODSFrom 2014 to 2018, 3 infants and 2 children (median age: 10 months; range: 6–40 months) with coronary artery anomalies underwent CABG at our hospital. The indications for the procedure included left main coronary artery atresia and stenosis in 2 and 3 patients, respectively. Graft patency was evaluated postoperatively by contrast-enhanced computed tomography or coronary angiography, and postoperative outcomes (including death and cardiac events) were assessed during the follow-up period.RESULTSNo 30-day or in-hospital mortalities were noted. Postoperative examinations revealed patent grafts in all patients. They were discharged without any cardiac complications. Regarding the outcomes at the follow-up period, the graft patency rate was 80.0% (4/5 grafts), with no deaths or cardiac events.CONCLUSIONSCABG is a useful strategy for coronary revascularization in infants and young children with coronary artery anomalies. Although the mid-term outcomes and patency are satisfactory, careful follow-up is necessary because the long-term outcomes remain unknown.     

Grafting an anomalous coronary artery: a left anterior descending from the right coronary artery grafted with the right internal thoracic artery

A left anterior descending artery (LAD) arising from the right coronary artery (RCA) or right sinus of Valsalva is an exceedingly rare anomalous variant occurring in 0.03% of the population. We here present the case of an 81-year-old male with severe triple vessel coronary artery disease who was found to have an aberrant LAD arising from the proximal RCA, and was successfully treated with off-pump, total arterial, and complete surgical revascularization.     

Double superior vena cava: presentation of two cases and review of the literature

Introduction: Various anomalies in the development of the great thoracic veins of the embryo can be incidentally discovered in the normal adult. Duplication of superior vena cava (SVC) is a rare abnormality, but the most common thoracic venous congenital anomaly.

Case reports-methods: We present two cases in the intensive care unit of our hospital, of asymptomatic patients who underwent an uneventful central line placement in the left subclavian vein. The track of the catheter, as shown in the X-ray, was misplaced to the left of the aorta and further investigation with computed tomography angiography confirmed a persistent left SVC. In both cases the vein drained into the coronary sinus and then to the right atrium. In the second case the echocardiography revealed a dilated coronary sinus.

Conclusions: Double SVC can be fortuitously discovered during catheter insertion, thoracic or cardiac imaging and surgery. In most cases it drains into the right atrium, through the coronary sinus. This entity is significant to the physician because of its importance in differential diagnosis as a cause of a widened mediastinum, as well as any difficulty that can occur in the placement of a central venous catheter or a pace maker.     

Open heart surgery in the first six months of life

Intracardiac repair of a variety of cardiac anomalies was performed in 104 infants aged 10 days to 6 mo, presenting with severe hypoxia and/or intractable heart failure. Thirty-eight patients (33 less than three mo of age) did not survive the operation or died during the first postoperative month. Above 6 mo of age, correction of heart defects is often carried out on an elective basis, and results are more favorable. A more convenient choice between corrective and palliative procedures is suggested to achieve better results in the difficult group of patients who require surgery within the first 6 mo of life.     

Adult congenital heart disease

For a decade now, it has been recognized that optimal management of adult congenital heart disease (ACHD) requires a skilled multidisciplinary team. The size and complexity of the population of adults with congenital heart disease (CHD) are increasing. This article reviews the general considerations for giving an anesthetic to an adult with CHD for cardiac or noncardiac surgery and provides further elaboration for a variety of complex patient types. Lastly, the advantages of an organized multidisciplinary approach to patients with ACHD are discussed.     

Giant aneurysm of the right atrial appendage repaired by minimally invasive approach

A 59‐year‐old female patient who was diagnosed with giant right atrial appendage aneurysm (75 × 87 mm) underwent minimally invasive repair via right mini‐thoracotomy. The aneurysm was completely excluded by linear method under beating heart without cardiac arrest. The postoperative recovery was uneventful and she was discharged home without symptoms 16 days after surgery.     

The significance of symptoms before and after surgery for anomalous aortic origin of coronary arteries in adolescents and adults

Open in a separate window OBJECTIVESThe aim of this study is to describe the significance of symptoms preoperatively and at medium-term follow-up in adolescent and adult patients who underwent surgery of anomalous aortic origin of a coronary artery (AAOCA).METHODSConsecutive patients who underwent surgery for AAOCA in our tertiary referral centre between 2001 and 2018 were included. Clinical characteristics and symptoms were evaluated and medium-term outcomes were recorded. Symptoms were classified according to the ‘2019 ESC guidelines on chronic coronary syndromes’.RESULTSA total of 53 (55% male) patients with mean age of 44 at time of surgery underwent surgical repair of AAOCA. Data on symptoms and events ˃3 months after surgery were available in 34 patients with a median follow-up of 3 years (interquartile range 1.0–5.3). Preoperatively, only 35% patients had typical anginal complaints. After surgical correction of AAOCA, 59% of the patients were free of symptoms, compared to 6% preoperatively (P < 0.001). A total of 3 (9%) patients needed a reoperation/reintervention related to the operated AAOCA. All 3 patients presented postoperatively with novel typical anginal complaints.CONCLUSIONSAdolescent and adult patients with AAOCA present with varying symptoms. Only 35% have typical anginal complaints. Surgical correction of AAOCA reduces the symptoms in the vast majority of patients. One should be aware of potential lesions of the operated coronary artery in patients presenting with typical anginal complaints postoperatively.     

Splenogonadal fusion: Report of a case and review of the literature

We report a case of splenogonadal fusion masquerading as a testicular tumor and review the etiology, the pathogenesis and the management of this rare disease. A 20-year-old male patient presented with clinical and radiological findings of a left testicular mass for which he underwent a left radical inguinal orchidectomy. Histopathology of the removed testicle revealed no tumor. The gonad was fused to ectopic splenic tissue, the later forming a distinct encapsulated mass attached to the lower pole of the testicle inside the tunica vaginalis. Splenogonadal fusion, although a rare condition, may account for a non-malignant testicular mass and should be suspected in young patients with other congenital anomalies.     

Understanding cardiac shunts

Most patients with congenital heart disease have a cardiac shunt whose direction and magnitude can have a major impact on cardiorespiratory physiology and function. The dynamics of the shunt can be significantly altered by anesthetic management and must be understood in order to provide optimal anesthetic care. Given that there are now more adults than children with congenital heart disease and that the majority of nonpediatric patients are cared for in centers without special expertise in congenital heart disease, it is imperative that all anesthesia providers have a general understanding of the subject. This educational review describes a technique to explain this complex subject using simple pictorial diagrams.     

Case report: airway and concurrent hemodynamic management in a neonate with oculo‐auriculo‐vertebral (Goldenhar) syndrome,severe cervical scoliosis,interrupted aortic arch,multiple ventricular septal defects,and an unstable cervical spine

We report the challenging case of a 1‐week‐old, term, 2.4 kg neonate with Goldenhar syndrome (including microcephaly, left microtia, left facial palsy, dextro‐scoliosis of the cervical spine, and cervico‐thoracic levoscoliosis), multiple ventricular septal defects, a type B interrupted aortic arch, a large patent ductus arteriosis, and radiographic and clinical signs concerning for an unstable cervical spine. Our anesthesia team was consulted for perioperative management of this patient during her surgical repair. This case report describes the use of the Air‐Q size 1 laryngeal airway (LA) to assist fiberoptic intubation in an ASA 4 neonate with cardiac disease, an anticipated difficult airway with the addition of an unstable cervical spine, as well as the anesthetic techniques used to maintain hemodynamic stability while the airway was secured.     

Left thorax approach to repair doubly committed juxta‐arterial ventricular septal defect with Da Vinci robotic system

Robotic repair of the ventricular septal defect was performed mainly for perimembranous type via right thorax approach. Minimally invasive strategies for doubly committed juxta‐arterial ventricular septal defect were limited. Here, for the first time, we successfully repaired a doubly committed juxta‐arterial ventricular septal defect with Da Vinci robotic system via left thorax approach. The technique could provide excellent exposure of surgical field and accurate repair, with the advantage of reducing trauma and shortening the overall length of stay.     

Pediatric cardiac surgery: an historical appreciation

Many of the early, classic pediatric cardiac surgical operations were named after their originators. Some of these continue to be performed in the original form, many in modified form and some are obsolete. The development of many of these important early operations is reviewed and they are placed in the context of their times.