Anomalous origin of the right coronary artery from the pulmonary artery

Anomalous origin of a coronary artery from the pulmonary artery is a relatively rarer congenital anomaly which almost exclusively involves the left coronary artery. A previously healthy 25-year-old woman had a cardiopulmonary arrest. Following resuscitation, investigations revealed an even rarer anomaly, an anomalous right coronary artery arising from the pulmonary artery. Successful surgical repair was accomplished by reimplanting the right coronary artery into the aortic root. The patient is currently asymptomatic 2 years postoperatively. The pertinent literature is reviewed.     

A case of total cavopulmonary connection with preceding tricuspid valve closure in pulmonary atresia and intact ventricular septum, right ventricular-coronary artery fistulae, and coronary artery stenoses]

More than half of the patients with pulmonary atresia and intact ventricular septum (PA/IVS) are known to complicate right ventricular-coronary artery fistula (fistulae) and particularly those with coronary artery stenoses bear a high mortality rate and remain in a surgical challenge. A 4-year-old girl was first admitted to our hospital at 5 days of age and right ventriculogram revealed markedly hypoplastic tripartite ventricle and multiple fistulae through which both coronary arteries and aortic root were retrogradely opacified. Echocardiographically measured diameter of the tricuspid valve was 5 mm (Z value: 4). She underwent pulmonary valvotomy and central aorto-pulmonary (AP) shunt at 16 days of age. Repeat right ventriculogram at 1.9 years of age disclosed multiple stenoses in left anterior descending coronary artery (LAD) with proximal dilatation and tortuosity. Additional findings of left ventricular dysfunction (LVEF of 61.5%) and depressed ST segment in left precordial leads prompted us to proceed to the second palliation which comprised take-down of central AP shunt, bidirectional cavopulmonary shunt and closure of tricuspid and pulmonary valves. Catheterization at 3.4 years of age disclosed antegradely filled LAD with apparent relief of stenoses and improvement of LVEF to 68.9%. She underwent definitive repair of total cavopulmonary connection at 4.0 years of age and is leading a normal life 2 years after surgery. This experience draws us to conclude that tricuspid valve closure is a meaningful palliative procedure for PA/IVS with fistulae and coronary artery stenoses, provided that proximal segments of both coronary arteries remain intact.     

Function of the rabbit supraspinatus muscle after detachment of its tendon from the greater tubercle. Observations up to 6 months

We successfully treated a case of active infective endocarditis in the remission phase of virus-associated hemophagocytic syndrome (VAHS). A 21-year-old man was admitted to our hospital for fever, arthralgia, and general fatigue. His blood cultures revealed staphylococcus epidermidis. He underwent urgent aortic valve replacement and closure of the abscess cavity because of an ineffective antibiotic therapy and a progressive left heart failure. Operative findings showed about 100 ml bloody pericardial effusion, fresh vegetation on the aortic left coronary and non-coronary leaflets, and aortic root abscess just below the left coronary ostium. The aortic root abscess extended to the left ventricular wall between the base of left atrial appendage and the base of main pulmonary artery and was in the state of impending rupture. The left main coronary artery was fully exposed after debridement in the abscess cavity. It was thought that left atrial appendage as a pedicle was useful for filling up the abscess cavity to protect infection.     

Anomalous origin of the left coronary artery from the pulmonary artery in an adult: tubular reconstruction of the left main coronary artery under coronary perfusion

A 38-year-old female with anomalous origin of the left coronary artery (LCA) from pulmonary artery was surgically corrected by tubular reconstruction of the left main coronary artery (LMCA) using the pulmonary artery wall, and this repair was performed under beating heart. Thus, the pulmonary artery was divided above the orifice level and just above the pulmonary valve, and the commissure between nonfacing and left side sinuses was dissected away from the pulmonary artery wall to obtain lateral flaps. The pulmonary artery defect was reconstructed with a roll using an autologous pericardial patch, while the detached commissure was suspended on the pericardial patch. The long tube constructed using pulmonary artery tissue was anastomosed to the anterior aspect of the ascending aorta. These procedures were performed under beating heart simply by clamping the LMCA, since the preoperative myocardial contrast echocardiography confirmed the adequate coronary collateral flow from the right circulation. The postoperative course was uneventful, and a coronary artery angiogram demonstrated a widely patent LMCA. Our experience suggests that, in adult cases, this procedure could be performed without myocardial ischemia simply by clamping the LMCA because of well-developed coronary collateral arteries. The safety of this technique could be confirmed by myocardial contrast echocardiography.     

Anomalous origin of the left coronary artery from the pulmonary artery (author's transl)

The case of a three year-old child with anomalous origin of the left coronary artery from the pulmonary artery is presented. He had a clinical picture and an electrocardiographic pattern of antero-lateral myocardial infarction that suggested a cardiac malformation. Once the diagnosis was established by cardiac catheterization and coronary arteriography, the defect was successfully correted by transplanting the anomalous coronary vessel to the aorta. The clinical, electrocardiographic and angiographic features of the anomaly are given.     

Abnormal branching of left pulmonary artery to the lateral and posterior basal segments

A 70-year-old male, underwent left lower lobectomy due to lung cancer, had an extremely rare form of left pulmonary artery branching. His pulmonary artery supplying to lateral and posterior basal segments (A9 + 10) was arisen anteriorly from the root of the left pulmonary artery in the mediastinum and ran below in the anterior portion of the upper lobe stem bronchus. Embriologically, this abnormality might be due to a derangement of anastomosis between the pulmonary arch system and splanic plexus in primitive lung, and was interpreted as one of the pulmonary sequestration. Clinically, particularly at thoracotomy, the presence of a large vessel in an unexpected location is very hazardous, so that preoperative pulmonary angiography is useful to detect the abnormal branching.     

Stenosis at the origin of an anomalous left main coronary artery arising from the pulmonary artery in a symptom-free adolescent girl: transesophageal echocardiographic findings

We report an unusual finding of ostial stenosis of an anomalous left main coronary artery originating from the pulmonary artery in a symptom-free adolescent girl. Transesophageal echocardiography with Doppler color imaging correctly identified all of the salient features of this anomaly including proximal stenoses of the right and left coronary arteries. These findings were subsequently confirmed at cardiac catheterization.     

Coronary artery fistula with left atrial myxoma: report of a case

A 64-year-old male was referred for surgical treatment of left atrial myxoma. Preoperative coronary angiography revealed coronary artery fistula from the left anterior descending artery and the circumflex artery draining into the main pulmonary artery. Operative treatment was performed including resection of the myxoma, patch closure of the atrial septal defect, and closure of the fistula with pledgeted mattress sutures from within the main pulmonary artery on cardiopulmonary bypass. His postoperative course was uneventful, and disappearance of the left atrial myxoma and the coronary artery fistula was ascertained by echocardiography and coronary angiography.     

Coronary-to-pulmonary artery shunts via the bronchial artery: analysis of cineangiographic studies

Coronary-to-pulmonary artery shunts via the bronchial artery (CA-BA-PA shunts) were observed in 16 of 2,922 consecutive patients who underwent selective coronary cineangiography. Underlying diseases included Takayasu arteritis (n = 8), chronic pulmonary inflammatory diseases (n = 4), pulmonary thromboembolism (n = 2), pulmonary artery tumor (n = 1), and tetralogy of Fallot with pulmonary atresia (n = 1). Ventilation-perfusion scans were available in 15 of the 16 patients. Mismatched defects were identified in 11 patients, and matched defects were identified in four. Bronchial-to-pulmonary artery shunts were detected on selective bronchial angiograms and/or thoracic aortograms in 13 patients. Feeding arteries of the CA-BA-PA shunts included left atrial branches (n = 13), right sinus node branches (n = 7), left sinus node branches (n = 2), right conal branch (n = 1), left conal branch (n = 1), and posterolateral branches (n = 2). These coronary branches seemed to serve as collateral vessels from a coronary arterial system with a higher pressure to a pulmonary arterial system with a lower pressure in conditions of decreased pulmonary flow or in cases of chronic pulmonary inflammatory disease. The importance of the coronary artery along with other superior thoracic collateral networks in contributing to the development of shunts to the pulmonary artery is underscored.     

Leiomyosarcoma of the pulmonary artery

Primary leiomyosarcoma of the pulmonary artery is an extremely rare tumor that is frequently misdiagnosed as chronic pulmonary embolism. In the present case, early diagnosis and location in the left pulmonary artery permitted resection by pneumonectomy with total cardiopulmonary bypass.     

A new operative technique for pulmonary artery banding: adjustment of pulmonary artery bands by mitral valve flow velocity

We developed a new technique to adjust the pulmonary artery band at surgery by monitoring the mitral valve flow velocity, which is indirectly indicative of the pulmonary flow. We employed this technique for 10 consecutive patients aged from 5 days to 5 months (mean, 1 months) weighing from 2.7 to 4.4 kg (mean, 3.3 kg). Underlying disease was aortic coarctation or interrupted+ventricular septal defect in 7 patients, single ventricule in 1 patients and miscellaneous defects in 2 patients. The pulmonary artery was exposed through a left lateral thoracotomy and a 3 mm wide Teflon tape was placed around the main pulmonary artery. The transducer of the Doppler echocardiography was placed along the left sternal border. The band was tightened gradually until the maximum velocity of the mitral valve flow decreased to around 70% of the previous level. During banding procedure, arterial oxygen saturation, heart rate and left ventricular contractility were monitored continuously. If bradycardia, unacceptable hypoxemia or ventricular dysfunction occurred, the band was released. The mitral valve flow velocity decreased rapidly by just a little additional tightness of the band between the range of 50% to 80% of the previous level. This technique enabled a very fine adjustment (less than 0.5 mm plication) and postoperative management has become very easy. Although there is a limitation of this technique that monitoring of the mitral valve flow velocity cannot be applied to the patients with significant interatrial shunt or mitral regurgitation, we conclude that this technique is simple and useful to obtain the optimum constriction of the pulmonary artery with excessive pulmonary blood flow.     

Left main coronary artery compression during primary pulmonary hypertension

Primary pulmonary hypertension (PPH) is often associated with angina-like chest pain, the mechanism of which is controversial. A 37-year-old woman with severe PPH and angina had transient ischemic ECG changes and reversible anterior perfusion defect on 201thallium scintigraphy. Coronary angiography revealed severe stenosis of the left main coronary artery (LMCA) and otherwise normal vessels. After heart-lung transplantation, examination of the explanted heart showed normal coronary arteries. Compression of the LMCA by the dilated pulmonary artery trunk was responsible for myocardial ischemia. This mechanism should be considered in patients with PPH and angina and might contribute to the high sudden death rate.     

Surgical treatment of coronary artery-pulmonary artery fistula

We experienced 4 cases of left coronary artery-pulmonary artery fistula. Two cases had small fistulas associated with atherosclerotic coronary lesions, and the other 2 had large fistulas with aneurysmal enlargement. In the former 2 cases, ligation of the fistulas and closure of the opening of fistula into the pulmonary artery through pulmonary arteriotomy were performed together with coronary artery bypass grafting and left ventricular aneurysmectomy. In one of the latter 2 cases, the fistula arising from the anterior descending branch was ligated and the opening of fistula draining into the pulmonary artery was closed through pulmonary arteriotomy. In another case, both openings of the fistula into the anterior descending branch and the pulmonary artery were closed from inside through incision of the dilated fistula. In all 4 cases, operations were performed using cardiopulmonary bypass and retrograde coronary perfusion, which could afford good heart protection even in cases with coronary lesions and coronary steal phenomenon. All cases went an uneventful postoperative course. Postoperative angiograms showed disappearance of the fistulas in 3 cases. In one case, however, residual fistula was found because a fine fistula might be overlooked. In such a case with complicated fistulas with aneurysmal enlargement, fistulas should be examined carefully through incision of the enlarged anomalous vessels. In this paper, diagnosis, operative indication and treatment for coronary artery-pulmonary artery fistula were discussed.     

Using outcomes data to compare plans, networks, and providers: what is the state of the art?

The short left coronary artery encountered during aortic root replacement with the modified Bentall (coronary button) technique may pose a significant problem for the surgeon. A simple solution entails the placement of a short interposition Dacron graft between the native coronary artery and the aortic graft. This approach has been successfully employed in 2 patients.     

pH sensitive properties of Tc(V)-DMS: analytical and in vitro cellular studies

Coronary arteries anomalies may be part of complex congenital malformations of the heart or be an isolated defect. In our anatomic collection of congenital heart disease, an isolated anomalous origin of coronary arteries was observed in 27 of 1,200 specimens (2.2%): left coronary artery from pulmonary trunk in five, origin from the wrong aortic sinus in 12 (both right and left coronary artery from the right sinus in four and from the left sinus in seven, left coronary artery from the posterior sinus in one), left circumflex branch from right aortic sinus or from very proximal right coronary artery in three, high takeoff of right coronary artery in three, stenosis of the coronary ostia attributable to valvelike ridge in four. In 16 (59%) patients (12 males and 4 females, age ranging from 2 months to 53 years; median, 14), the final outcome was sudden death; it occurred in all cases of left coronary artery origin from right aortic sinus, in 43% of right coronary artery origin from left aortic sinus, and in 40% of the left coronary artery from the pulmonary trunk. Sudden death was precipitated by effort in eight (50%) and was the first manifestation of the disease in eight (50%); previous symptoms consisted of recurrent syncope in four, palpitations in three, and chest pain in one. Five patients who died suddenly during effort were athletes. In conclusion, (1) more than half of our postmortem cases with anomalous origin of coronary arteries died suddenly, (2) all but two patients with sudden death had anomalous coronary artery origin from the aorta itself, (3) the fatal event was frequently precipitated by effort, (4) palpitations, syncope, and ventricular arrhythmias were the only prodromic symptoms and signs. Recognition during life of these coronary anomalies, by the use of noninvasive procedures, is mandatory to prevent the risk of sudden death and to plan surgical correction if clinically indicated.     

Aortic dissection extending from the left coronary artery during percutaneous coronary angioplasty

A 72-year-old woman with acute aortic dissection as a complication of percutaneous coronary angioplasty was successfully treated. She received a graft replacement of the ascending aorta as well as triple coronary artery bypass grafts. The dissection had extended from the left coronary artery. Although acute aortic dissection is a rare complication of percutaneous coronary angioplasty, physicians and cardiac surgeons should keep its potential occurrence in mind.     

Anomalous coronary artery with tetralogy of Fallot and aortopulmonary window

Anomalous origin of the left main coronary artery from the pulmonary artery is rarely associated with other conditions. We report the case of an infant born with tetralogy of Fallot and aortopulmonary window who at the time of surgical repair was found to have an anomalous left main coronary artery originating from the right pulmonary artery.     

Sudden unexpected death resulting from an anomalous hypoplastic left coronary artery

We present a case of sudden death in a 24-year-old, healthy white female who was physically active and participated in sports, including soccer. Two weeks prior to her death, an insurance physical examination revealed an abnormal electrocardiogram which demonstrated flipped T waves in the anterior leads. There was no other remarkable medical history. At autopsy, only one coronary ostium was demonstrated and it originated from the right aortic sinus. Approximately 0.8 cm from this right coronary artery (RCA) ostium, a left coronary artery (LCA) branched off the RCA at a 90-degree angle. The LCA had luminal diameter of 0.4 m but the LCA had a luminal diameter of only 0.1 cm. The LCA coursed anterior to the base of the pulmonary artery and down the anterior ventricular septum reflecting the usual course of the left anterior descending (LAD) coronary artery. The LCA and RCA paths appeared to merge or terminate at the anterior left ventricular myocardium which was discolored gray, a process that involved the inner and middle thirds of the myocardium. Based on the autopsy findings, we certified the cause of death as a probable arrhythmia due to myocardial fibrosis and dystrophic calcification resulting from complications of an anomalous hypoplastic left coronary artery. Anomalies of coronary arteries have been classified and studied at autopsy and by clinical angiography. Coronary artery anomalies can be divided into minor and major forms with major anomalies often resulting in cardiac dysfunction that may cause failure and death. Minor anomalies, in general, have no pathophysiological significance and are compatible with life. Minor anomalies include variations in number and location of coronary ostia. A single coronary ostia is exceedingly rare in hearts with no other congenital malformations. The prognostic significance can be unpredictable. A single coronary artery has the potential to be dangerous if obstructed at its main stem, or if it branches at an acute angle. Additionally, hypoplasia of one or more coronary arteries has been found to be associated with sudden death.     

Sub-trochanteric fractures. A comparative study between gamma nail and angular osteosynthesis with lateral cortical support]

METHODS: Thirty-three children and young adults with congenital aortic valve disease underwent pulmonary autograft replacement of the aortic valve between October 1993 and March 1997. There wer six females and 27 males; at operation, median age was 16 years (range: 3 to 41 years) and median body weight 60 kg (range: 14 to 121 kg). Fifteen patients (46%) had undergone one or more previous cardiac surgical procedures. A bicuspid aortic valve was present in 31 patients (94%); moderate to severe aortic stenosis and regurgitation was present in 10 (30%) and 26 (79%), respectively. RESULTS: All patients underwent the Ross procedure while in NYHA class I (64%) or class II (36%). A preoperative shortening fraction of 41 +/- 1.4% suggested well-preserved systolic function, but the mean left ventricular end-diastolic pressure of 16.6 +/- 1.3 mmHg was consistent with preoperative left ventricular pressure and volume overload. The aortic root was replaced using an interrupted suture technique in two patients and with three separate running sutures in 31. The right ventricular outflow tract was reconstructed in all classes with a cryopreserved pulmonary homograft valved conduit (median diameter 23 mm; range 19 to 30 mm). Intraoperative complications included transient atrioventricular dissociation (one), permanent atrioventricular dissociation (one), and left coronary artery distortion relieved by shortening the distal ascending aorta (one). Postoperatively, postpericardiotomy syndrome developed in six patients (18%), supraventricular tachycardia in three (9%), and ventricular tachycardia in one (3%). At three days after surgery, one patient developed ischemic left ventricular dysfunction requiring repositioning of the distorted left coronary artery higher on the neo-aortic root. Hospital survival rate was 100%. During a median follow-up of 17 months (range: 1 to 41 months) one patient suffered a non-cardiac death due to blunt trauma. there has been a significant postoperative improvement in NYHA class among surviving patients (class I, 94%; class II, 6%; p = 0.004 versus preoperative). Postoperative aortic regurgitation was absent or trivial in 17 (60%) and mild in the remaining 11 (40%) patients for whom follow-up echocardiographic data are available. One patient required reoperation 16.5 months after the Ross procedure to replace a rapidly degenerating pulmonary homograft, and one with moderately severe homograft stenosis and five with mild homograft stenosis are being monitored. Postoperatively, a gradual early expansion in the diameter of the neo-aortic root and reduction in echocardiographic indices of left ventricular hypertrophy and dilatation occurred. CONCLUSIONS: Pulmonary autograft replacement of the aortic valve in young patients with congenital aortic valve disease has produced excellent short-term anatomic/physiologic results and symptomatic relief with no mortality. Indices of left ventricular dilatation and hypertrophy regress after repair when the Ross operation precedes important deterioration in preoperative ventricular function. Important technical considerations include: (i) the native distal ascending aorta should be sufficiently shortened before performing the distal aortic anastomosis; and (ii) the left coronary anastomosis should be positioned relatively high on the neo-aortic root with a slight amount of tension. Both of these maneuvers reduce the likelihood of coronary artery distortion. Rapid degeneration of the pulmonary homograft and the propensity towards progressive dilatation of the neo-aorta are important postoperative considerations. Until more is known about the etiology and natural history of these two potential complications, postoperative anti-inflammatory and/or immunosuppressive therapy and strict control of hypertension should be strongly considered.     

Anomalous origin of the left coronary artery from the pulmonary artery in adults. Long-term follow up

Anomalous origin of the left coronary artery from the pulmonary artery is a serious pathology, that untreated supposes a high mortality. Usually it becomes symptomatic in early infancy, but some cases reach adult life with no symptoms. We report four cases diagnosed in teenagers and adults, three of them with the oldest ages known in worldwide literature and one of them the oldest reported until now. We have completed a long follow-up in the various surgical techniques, where we did not observe any differences in their prognosis, and we recommend an anti-arrhythmia treatment joined with surgical treatment in adult patients with this pathology.