Anomalous origin of left coronary artery from the pulmonary artery does the management in the adult differ from that of the infant? Four cases of the Bland-White-Garland Syndrome

We report four adult patients with anomalous origin of left main coronary artery from the pulmonary artery that presented to us from 1995 to 2006. One died suddenly at the age of 21 years before undergoing surgery, two patients had ligation and left internal mammary artery (LIMA) grafting to the left anterior descending artery, and one had aortic implantation of the left main coronary artery. All three patients who had surgical treatment are doing well.     

Anomalous Left Coronary Artery from the Pulmonary Artery with a Large Patent Ductus Arteriosus: Aversion of a Catastrophe

We present an infant who had an anomalous left coronary artery arising from the pulmonary artery (ALCAPA) and a large patent ductus arteriosus (PDA), who was diagnosed before a potentially catastrophic closure of PDA. In the presence of normal left ventricular function and the absence of coronary artery collaterals, it is difficult to diagnose ALCAPA. A disproportionate degree of left ventriclular dilation and severity of mitral valve regurgitation relative to the degree of PDA shunt, and echogenic papillary muscles on an echocardiogram should raise a suspicion of coronary artery anomalies. The infant underwent surgical ligation of PDA with translocation of coronary arteries and had an uneventful recovery.     

Radiofrequency recanalization of an occluded long segment left pulmonary artery in bidirectional cavopulmonary anastomosis

In patients with hypoplastic left heart syndrome who experience the Norwood surgical palliative program, development of the pulmonary artery branches is crucial. Balloon dilation and stenting may relieve potential stenosis but complete occlusion remains difficult to address with transcatheter therapy. We report the use of radiofrequency followed by balloon dilation and stenting to treat a long segment left pulmonary artery occlusion in a 15‐month‐old boy bidirectional Glenn. The left pulmonary artery was successfully recanalized with an excellent short‐term outcome. This further enhances the interest of radiofrquency for recanalization of occluded pulmonary artery branches, as an alternative to surgical reconstruction. © 2009 Wiley‐Liss, Inc.     

A rare case of anomalous origin of the left anterior descending artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease, with an incidence of <1 in 300 000 live births. We describe a rare case of a 23‐month‐old infant admitted for cough and fever. Echocardiography showed a branch of the left coronary artery originating from the pulmonary artery. The coronary computed tomographic angiography revealed anomalous origin of the left anterior descending (LAD) artery from the pulmonary artery with the left circumflex artery and right coronary artery arising normally from the aorta. The infant successfully underwent surgical reimplantation of the LAD to the ascending aorta. Our case emphasizes that echocardiography and computed tomographic angiography are valuable imaging modalities for making an accurate diagnosis and determining the precise surgical plan.     

Pulmonary atresia, VSD in association with coronary-pulmonary artery fistula

Congenital coronary-pulmonary artery fistula is rare in patients with pulmonary atresia and ventricular septal defect. The nomenclature, physiological, clinical, and surgical implications of these fistulas are yet to be defined. We report a one-year-old child with pulmonary atresia, ventricular septal defect, and a right coronary-pulmonary artery fistula who also had a diminutive, disconnected left pulmonary artery in addition to aortopulmonary collaterals. The patient underwent corrective surgery. However, the fate of diminutive pulmonary arteries is unknown. The literature was reviewed to explore the clinical or surgical implications of such fistulas for improved understanding and management in the future.     

Anomalous origin of the left pulmonary artery from the ascending aorta. Successful surgical correction in an infant with Fallot's tetralogy

We describe the case of a 40-day-old female patient with a history of breathlessness since birth who was referred to our hospital for surgical correction of common arterial trunk. The invasive investigation disclosed a Fallot cent s tetralogy anatomy associated with an anomalous origin of the left pulmonary artery from the ascending aorta. Immediately after diagnosis, the patient underwent a successful total surgical correction of the defect, including simultaneous anastomosis of the left pulmonary artery to the pulmonary trunk.     

Anomalous origin of the left coronary artery from the pulmonary trunk in a 45-year-old woman

We report a rare case of anomalous origin of the left coronary artery from the pulmonary trunk in a 45-year-old woman. The approach and technique used for selective catheterization of an anomalous left coronary artery arising from the pulmonary trunk are described. Six years after diagnosis, echocardiography showed left ventricular disfunction, and surgical treatment was indicated again. The origin of the left coronary artery from the pulmonary trunk was closed, and the postoperative period was uneventful, with recovery of left ventricular function and disappearance of ischemic features on stress myocardial perfusion imaging with 99m Tc-sestamibi, performed 4 weeks after surgery.     

Complex pulmonary atresia in a neonate: successful surgical palliation for right pulmonary artery arising from the right main coronary artery

We describe the case of a 3-week-old infant who was diagnosed with pulmonary atresia, a ventricular septal defect, and discontinuous pulmonary arteries. The pulmonary artery anatomy was delineated by angiography: the right pulmonary artery arose from the proximal right coronary artery, and the left pulmonary artery arose from a left ductus arteriosus. This case illustrates the importance of careful delineation of the anatomy in complex pulmonary atresia before surgical intervention.     

An adult case of Bland-White-Garland syndrome with collaterals from the bronchial artery

Summary We report herein an adult case of anomalous origin of the left coronary artery from the pulmonary artery (Bland-White-Garland syndrome; BWG syndrome). The patient had chest discomfort during exercise and signs of mitral regurgitation. We diagnosed him as having BWG syndrome by angiography, and performed surgical treatment. At the operation, retrograde coronary blood flow was found through the left coronary artery during aortic crossclamping. This suggested extracardiac anastomosis from peripheral arteries to the left coronary artery. Since the collateral blood flow was considerable during aortic cross-clamping, only the left coronary artery was closed. A collateral from the left bronchial artery to the left circumflex artery was demonstrated by postoperative angiography. Cases of adults with BWG syndrome are rare, and this may be the first report of a collateral from the bronchial artery.     

Pulmonary arterial embolus by an unusual wandering bullet

We report on a wandering bullet embolus to the left pulmonary artery after it had first passed from the right ventricular to the right renal vein via the inferior vena cava. Its presence in the left pulmonary artery was confirmed by pulmonary angiography. Hemorrhage due to the right ventricular wound was controlled by a median sternotomy and the bullet was extracted by left lateral thoracotomy. Intravascular migratory bullets continue to be a surgical curiosity. Clinical diagnosis may present a difficult aspect in emergency practice and angiography is mandatory. The removal of foreign bodies is recommended by the majority of authors.     

Surgical correction for one pulmonary artery arising from ascending aorta--report of five cases

Over a 10-year period we encountered 5 infants with a pulmonary artery branch arising from ascending aorta. Surgical re-implantation of this vessel was carried out at ages 2.5, 5, 8, 9, and 19.5 months. Pre-operative cardiac catheterization demonstrated severely raised pulmonary artery pressures in all, equal to systemic in 3, and suprasystemic in 2. Four patients had origin of the right pulmonary artery from ascending aorta with a left aortic arch, and the remaining patient had an anomalous left pulmonary artery associated with a right-sided aortic arch. All patients had substantial reduction in pulmonary artery pressures immediately following surgery. One patient died 18 days post-operatively from extensive lung disease. In all 4 of the survivors, post-operative cardiac catheterization (11 to 85 months after surgery) has shown a drop in pulmonary artery pressures. One patient has been left with mildly elevated systolic values but normal diastolic levels. In 2 of the children, mild stenosis has been found at the site of reimplantation of the pulmonary artery. This anomaly should always be considered as a cause in the setting of a large left to right shunt with tricuspid incompetence and severe right ventricular hypertrophy. Prompt surgical repair, after confirmation of the diagnosis, should prevent death from heart failure or the development of irreversible pulmonary vascular disease.     

Isolated origin of the left subclavian artery from the left pulmonary artery

We describe two children with isolated origin of the left subclavian artery from the left pulmonary artery detected by echocardiography during the assessment of their congenital cardiac malformations. Both patients demonstrated pre-operative evidence of subclavian steal. This entity results from persistence of the dorsal segment of the sixth left arch, with regression of the left fourth arch and interruption of the left dorsal arch distal to the origin of the seventh left intersegmental artery. The significance of this finding relates to the potential for pulmonary overcirculation, which could have significant post-operative ramifications if not detected prior to surgical repair of an associated cardiac malformation. This entity differs from cases with a right aortic arch and aberrant left subclavian artery which has the potential to form a vascular ring, unlike cases with isolated origin of the left subclavian artery from the pulmonary artery that do not cause compression of the airway.     

Adult‐type ALCAPA syndrome: A rare coronary artery anomaly

Anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA or Bland–White–Garland syndrome) is a rare but serious congenital coronary artery anomaly, with a poor prognosis without surgical repair. There are two types of ALCAPA syndrome: infant type and adult type. We present a rare case of a 63‐year‐old female patient, with isolated left anterior descending artery origin from the pulmonary artery. Coronary computed tomography angiography revealed giant and tortuous coronary arteries with many collaterals between the left and right coronary system. The patient refused any surgical treatment.     

Ductus arteriosus associated with an anomalous left coronary artery arising from the pulmonary artery: catastrophe after duct ligation.

Chronic left ventricular failure developed two months after ligation of an apparently uncomplicated large ductus arteriosus in a one year old girl. Two years later deterioration had progressed to a terminal stage. An anomalous left coronary artery arising from the pulmonary artery had not been recognised before duct ligation. This anomaly was diagnosed two years after ligation and was then treated by surgical redirection, but the patient died of severe left ventricular dysfunction.     

Successful percutaneous coil embolization of coronary–pulmonary, –carotid, and –internal mammary artery fistulas

We herein describe a 57-year-old man with coronary?Cpulmonary artery fistulas that had abnormal connections between the left common carotid artery and the left internal mammary artery. The patient was treated with percutaneous coil embolization using antegrade (via the coronary artery) and retrograde (via the pulmonary artery) approaches. Coronary artery fistulas have diverse anatomical variations, and it is important to thoroughly evaluate the anatomy before beginning any mode of treatment, surgical or endovascular. In the case reported herein, multislice computed tomography played a pivotal role in the preprocedure evaluation.     

Complete obstruction of the left main coronary artery associated with congenital pulmonary valvular stenosis.

The surgical treatment of the completely obstructed left main coronary artery is aortocoronary saphenous-vein bypass to the left coronary artery system. Without surgical intervention, this lesion has an exceedingly poor prognosis. This report describes a patient with atherosclerotic obstruction of the left main coronary artery and congenital pulmonary valvular stenosis, an unusual combination of lesions not previously documented together. The patient remains asymptomatic 22 months after aortocoronary saphenous-vein bypass and pulmonary valvotomy.     

Ductus arteriosus associated with an anomalous left coronary artery arising from the pulmonary artery: catastrophe after duct ligation

Chronic left ventricular failure developed two months after ligation of an apparently uncomplicated large ductus arteriosus in a one year old girl. Two years later deterioration had progressed to a terminal stage. An anomalous left coronary artery arising from the pulmonary artery had not been recognised before duct ligation. This anomaly was diagnosed two years after ligation and was then treated by surgical redirection, but the patient died of severe left ventricular dysfunction.     

Partial anomalous origin of the left pulmonary artery

The pulmonary sling or aberrant left pulmonary artery has an incidence of 3% to 6% of all anomalies of the aortic arch system. We report a boy with coexistence of a normal and an anomalous left pulmonary artery. Associated congenital anomalies were coarctation, mitral stenosis, and imperforate anus. Cardiac catheterization and angiocardiography at the age of 5 months demonstrated an inconspicuous pulmonary trunk dividing into left and right branches. There was a normal right pulmonary artery, and a main left pulmonary artery supplying the left upper lobe, lingula, and anterior segments of the left lower lobe. In addition, an anomalous left lower lobe artery originated from the proximal right pulmonary artery, passed inferior to the tracheal bifurcation to the left supplying posterior segments of the left lower lobe. Chest X-rays and bronchoscopy could not detect any malformation of the trachea and pulmonary lobulation. There are only three reports of partial anomalous origin of the left pulmonary artery, and only one had a similar course of the accessory artery. This report is the first to present selective angiography and echocardiographic findings of the partial anomalous left pulmonary artery. The development of the partial anomalous pulmonary artery can be explained by the plexiform nature of the primary pulmonary vascular bed and can help to understand the embryology of the pulmonary arteries.     

Plexus network between internal mammary graft and pulmonary vasculature after coronary bypass grafting

Left internal mammary artery to pulmonary vasculature fistulas are rare complications following coronary artery bypass grafting surgery. In symptomatic cases, management may either be conservative, or involve coil embolisation or surgical ligation of the fistula. We describe a 52-year-old male patient who suffered from an acute coronary syndrome due to significant stenosis of the right coronary artery 12 years after coronary artery bypass grafting surgery. Coronary angiography accidentally revealed a fistulous connection between the left internal mammary artery and the left pulmonary vasculature. The patient underwent percutaneous coronary intervention for his right coronary artery and was managed conservatively for his fistula.     

The development of a pseudo-chamber after balloon pulmonary angioplasty: long-term complications of balloon pulmonary angioplasty

We experienced a rare complication where extravasation developed a pseudo-chamber long after the balloon pulmonary angioplasty for supravalvular pulmonary stenosis. A 3-month-old girl was diagnosed with an anomalous origin of the left coronary artery from the pulmonary artery. She underwent the Takeuchi procedure at 10 months of age. During the follow-up, the supravalvular pulmonary stenosis deteriorated, and was treated by balloon pulmonary angioplasty with the double balloon technique catheter at 6 years of age. Angiography at the main pulmonary artery showed a small amount of extravasation contrast medium after the procedure. Follow-up echocardiography showed a diminished extravasation hemorrhage. Twelve years later, right ventricular enlargement due to pulmonary regurgitation had been observed on echocardiography. In addition, abnormal echo free space was detected at the left posterior of the left atrium. Enhanced computed tomography clearly demonstrated there was an orifice and extent of the pseudo-chamber. Surgical findings revealed a large tear just distal to the coronary tunnel. We speculated that extravasation blood was limited in the perivascular area early after the procedure but eventually reached the non-adhesive oblique pericardial sinus with age. Consequently, pulmonary to oblique pericardial sinus communication was established and looked like a pseudo-chamber long after the procedure. In conclusion, even if extravasation seems to be limited immediately after the balloon pulmonary angioplasty, it could expand for non-adhesive space and could develop a huge blood space like chamber. Long-term careful observation should be necessary for extravasation of pulmonary artery even with surgical adhesion.