Surgical correction of congenital left coronary-pulmonary artery fistula and rheumatic mitral valve disease. Case report

A 56-year-old woman with stenosis and incompetence of the mitral valve and clinical signs of congestive heart failure was found to have a communication between the left anterior descending coronary artery and the pulmonary trunk. A mitral valve prosthesis was inserted and the fistula was closed from within the pulmonary artery.     

Cleft mitral valve in association with anomalous left coronary artery arising from pulmonary artery

An anomalous origin of the left coronary artery from the pulmonary artery is commonly associated with mitral valve insufficiency. Usually this is secondary to left ventricular dysfunction or papillary muscle ischemia. We describe the association of an anomalous left coronary artery from the pulmonary artery with structural abnormalities of the mitral valve (cleft mitral leaflet) in 2 children, both of whom were being investigated for primary mitral valve disease. Both underwent successful operations for coronary transfer and mitral valve repair.     

Surgical treatment of a pulmonary artery aneurysm

We would like to report a rare case of post-stenotic aneurysm of the pulmonary trunk and its left branch in a 51-year-old man. His cardiac disease, which was first diagnosed at the age of 4, was left untreated because of absence of symptoms and normal physical development. A CT scan, recently performed because of decrease in exercise tolerance and worsening dyspnea, showed a pulmonary artery aneurysm (52 mm × 79 mm). The echocardiography revealed a severe pulmonary commissural stenosis. Through a surgical approach the pulmonary trunk and its left branch were excised and reconstructed using a 30 mm Dacron graft; the right pulmonary branch was then reimplanted on the right side of the tube. The patient's postoperative course was uneventful. He was discharged on the seventh postoperative day and there were no adverse events or complications at 1- and 3-month follow-up.     

Anomalous left coronary artery from pulmonary artery with mitral stenosis

The usual presentation of anomalous left coronary artery from pulmonary artery is severe left-sided heart failure and mitral valve insufficiency presenting during the first months of life. The manifestations of left heart failure may be masked if pulmonary artery pressure remains high. We believe this is a rarest of rare case of anomalous left coronary artery from pulmonary artery with severe mitral stenosis and pulmonary hypertension in which pulmonary hypertension, along with good collateral circulation helped to preserve left ventricular function.     

The anatomy of the heart

The normal heart is the size of the patient's closed fist. The venae cavae drain into the right atrium, which bears the fossa ovalis and receives the coronary sinus and the anterior cardiac vein. The atrium empties into the right ventricle through the tricuspid valve. Both ventricles have trabeculated walls (trabeculae carneae), and from some project the papillary muscles, bearing the chordae tendinae attached to the free borders of the tricuspid valve. The same arrangement is seen on the left side. The right ventricle leads to the pulmonary trunk, guarded by its three valve cusps. Oxygenated blood returns to the left atrium via the four pulmonary veins and passes to the left ventricle via the mitral valve. Exit is through the tricuspid aortic valve. The right and left coronary arteries arise above the valves, their orifices lying in the sinuses of Valsava. The right coronary artery lies in the right part of the atrioventricular groove and gives off the posterior interventricular artery. The left coronary arteries divide into the anterior (descending) interventricular branch and the circumflex branch. Major veins accompany the arteries, except for the anterior cardiac vein, which drains directly into the right atrium.     

The anatomy of the heart

The normal heart is the size of the patient’s closed fist. The venae cavae drain into the right atrium, which bears the fossa ovalis and receives the coronary sinus and the anterior cardiac vein. The atrium empties into the right ventricle through the tricuspid valve. Both ventricles have trabeculated walls (trabeculae carneae), and from some project the papillary muscles, bearing the chordae tendinae attached to the free borders of the tricuspid valve. The same arrangement is seen on the left side. The right ventricle leads to the pulmonary trunk, guarded by its three valve cusps. Oxygenated blood returns to the left atrium via the four pulmonary veins and passes to the left ventricle via the mitral valve. Exit is through the tricuspid aortic valve. The right and left coronary arteries arise above the valves, their orifices lying in the sinuses of Valsava. The right coronary artery lies in the right part of the atrioventricular groove and gives off the posterior interventricular artery. The left coronary arteries divide into the anterior (descending) interventricular branch and the circumflex branch. Major veins accompany the arteries, except for the anterior cardiac vein, which drains directly into the right atrium.     

An alternative procedure for correction of anomalous origin of left coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital cardiac malformation that is commonly associated with mitral insufficiency. Direct implantation of the anomalous origin of the left coronary artery from the pulmonary artery into the aorta is ideal, but it may not be fit for some patients whose anomalous left coronary arteries arise remotely from the ascending aorta. To solve the same technical problem in a boy with anomalous origin of the left coronary artery from the pulmonary artery in combination with moderate mitral insufficiency, we successfully elongated the anomalous coronary artery by creating a tube-shape graft using part of the pulmonary arterial wall. Simultaneous mitral annuloplasty was performed after a dual-coronary repair.     

Atherosclerosis in type IV dual left anterior descending artery and anomalous aortic origin of the left circumflex artery in association with rheumatic valve disease: a case report

Double left anterior descending coronary artery arising from the left and right coronary arteries is a very rare congenital coronary artery anomaly. In this case, there was also a circumflex artery arising from the right sinus Valsalva and in association with severe rheumatic valve disease. Subsequently, the patient underwent mechanical aortic valve replacement with a 21-mm bileaflet mechanical aortic valve and coronary artery bypass grafting. We performed coronary artery bypass grafting of 3 vessels, including the left internal mammary artery to the large diagonal branch and the saphenous vein graft to the circumflex artery and the right coronary artery, under cardiopulmonary bypass. In this report, we describe an unusual case of this combination in association with both atherosclerosis and rheumatic aortic and mitral valve disease.     

A case of pulmonary arteriovenous fistula with mitral stenosis

The patient was a 61-year-old female, complaining of cyanosis, dyspnea and shortness of breath on exertion. She was diagnosed as having a pulmonary arteriovenous fistula (PAVF) in combination with mitral stenosis. The fistula was located in the left lower lobe and a right-left shunt of 28.7% was detected. Cardiac catheterization showed a pulmonary artery pressure of 44/22 mmHg (mean pressure, 31 mmHg). By occluding the PAVF using a balloon catheter, PaO2 increased from 47 mmHg to 88 mmHg. The mitral stenosis of this patient was though to be a mild form, and PAVF seemed to be responsible for symptoms. Since left lobectomy together with mitral valve replacement was considered to have a high risk, left lower lobectomy was performed initially. Thereafter mitral valve replacement was done successfully. Separate operations for PAVF and mitral stenosis are likely to be beneficial in patients with mitral stenosis associated with moderate pulmonary hypertension.     

Treatment with epoprostenol of pulmonary arterial hypertension following mitral valve replacement for mitral stenosis

Pulmonary hypertension frequently complicates mitral stenosis. Increased pulmonary artery pressure results from raised left atrial pressure, pulmonary arteriolar constriction, and obliterative changes in the pulmonary vascular bed, and usually responds to surgical relief of mitral stenosis. However, severe pulmonary hypertension may persist after surgical treatment of mitral stenosis. We describe a patient whose severe pulmonary hypertension following mitral valve replacement was treated successfully with continuous intravenous epoprostenol.     

A novel view for visualizing a left pulmonary artery thromboembolus with intraoperative transesophageal echocardiography

Visualization of thromboembolic material in the pulmonary artery is often difficult on transesophageal echocardiography, especially in the left pulmonary artery, because of the position of the left main bronchus. We present a case in which thromboembolic material within the midleft pulmonary artery was incidentally diagnosed using additional, modified transesophageal echocardiography views, in a patient undergoing mitral valve repair.     

Anomalous origin of the left coronary artery from the pulmonary artery: new electrocardiographic, echocardiographic and surgical observations

The case of a two year old girl with anomalous origin of the left coronary artery from the pulmonary artery is described. She was never in heart failure but had cardiomegaly and anginal pain. The ECG showed a typical infarct pattern with left ventricular hypertrophy. An unusual finding was a prolonged QTc of 0.52. During cardiac catheterization and twice 24 hours later she developed ventricular fibrillation treated with electroshock and prevented later with propranolol. The QTc returned to normal after surgery. Echocardiography showed diastolic flutter and early systolic closure of the pulmonary valve. This disappeared after surgical correction. Transverse 2D echo of the aortic root showed a large right coronary artery which decreased in size after surgery. The left coronary artery was not seen on echocardiography. At cardiac catheterization the diagnosis of an anomalous origin of the left coronary artery from the pulmonary artery was established, with a large shunt to the pulmonary artery through the anomalous artery. Mild pulmonary hypertension and mild mitral regurgitation were present. At surgery, since direct implantation was technically impossible, the left coronary artery was successfully connected to the aorta via a 6 mm expanded Poly-Tetra-Fluoro-Ethylene (P.T.F.E.) graft.     

New technique for enlargement of the pulmonary outflow tract in corrected transposition of the great arteries [SLL]--a report of 2 cases

New technique for enlargement of the pulmonary outflow tract was performed in two patients with corrected transposition of the great arteries [SLL] associated with atrial septal defect, ventricular septal defect, pulmonary stenosis and mitral regurgitation. The middle of the anterior leaflet of the mitral valve was incised to the valve annulus towards the mid-point of the mitral-pulmonary fibrous continuity. In this approach, anterior node and anterior atrioventricular conduction bundle were securely protected from the surgical incision. The pulmonary annulus was divided posterolaterally and the incision was further extended into the pulmonary trunk to the bifurcation. The pulmonary trunk was enlarged with a fusiform patch of the xenogenous pericardium bearing monocusp. In case 1, St. Jude Medical valve #31 was implanted in the mitral position. In case 2, mitral valvular annuloplasty with Carpentier ring #36 which was deformed to admit enlarged portion of the pulmonary trunk. The VSD was closed through the right atrium, placing the suture on the left side of the septum. However, complete A-V block ensured temporarily due to retraction at the operation in case 1. No conduction disturbance ensured in case 2. This technique can provide some advantage in avoidance of injuries to the anterior node and the anterior atrioventricular conduction bundle. Application of this technique to the corrected transposition of the great arteries without mitral regurgitation is to be further evaluated.     

Left coronary artery originating from the pulmonary artery correction and total myocardial blood flow measurements

In a boy aged 12 years correction of an anomalous coronary artery system was performed by suture of the left coronary artery origin at the pulmonary artery trunk and a vein by-pass to the proximal LAD. Peroperative blood flow gives values for myocardial perfusion before and after by-pass with relation to patency of the vein graft. Before correction pure coronary blood flow was 540 ml/min, shunt flow 540 ml/min; in total 1,080 ml/min through the right coronary artery. After correction the vein by-pass conducted 310 ml/min and the right coronary artery 180 ml/min. At follow-up 2 months later coronary angiography showed a normal right coronary artery without collateral connections to the left coronary artery system which was well perfused from the by-pass. Left heart hypertrophy was reduced from 568 ml/m2. A weak systolic murmur at the apex could be a slight unimportant mitral insufficiency.     

The early course of pulmonary artery hypertension in patients undergoing mitral valve replacement with cardioplegic arrest

In an effort to describe the immediate course of pulmonary hypertension following mitral valve replacement, we reviewed preoperative and postoperative data from 62 patients who underwent mitral valve replacement. Patients were divided based on the absence (Group I) or presence (Group II) of severe preoperative pulmonary hypertension, defined as a mean pulmonary artery pressure greater than or equal to 40 mm Hg. Group II patients were subdivided based on the absence (Group IIa) or presence ( Group IIb) of markedly elevated preoperative pulmonary vascular resistance indices, defined as a greater than or equal to 700 dynes . sec . cm-5 . m2. Pulmonary artery wedge pressures fell promptly following mitral valve replacement in all groups, but the course of other hemodynamic parameters varied among groups. Cardiac index increased significantly among Group I and IIb patients but not among Group IIa patients. Group I patients did not have significant changes in mean pulmonary artery pressure and pulmonary vascular resistance index. Group IIa patients had substantial reductions in mean pulmonary artery pressure while pulmonary vascular resistance index remained near 400 dynes . sec . cm-5 . m2. Group IIb patients had substantial reductions in mean pulmonary artery pressure while pulmonary vascular resistance index fell significantly to about 400 dynes . sec . cm-5 . m2. Primary valvular lesion and pharmacologic support were insignificant variables. Data from these hemodynamic groups suggest that at least three mechanisms contribute to the pulmonary hypertension seen in mitral valve disease: passive transmission of elevated left atrial pressures, reactive pulmonary arteriolar vasoconstriction, and morphologic changes in the pulmonary vasculature. The first two mechanisms appear to be rapidly reversed following mitral valve replacement. While others have described the regression of pulmonary hypertension several months following mitral valve operations, data presented here suggest that changes in pulmonary artery pressures and pulmonary vascular resistance index may occur much earlier.     

A reoperation of adult-Type Bland-White-Garland syndrome

We present a 25-year-old female patient with Bland-White-Garland syndrome (anomalous origin of the left coronary artery from the pulmonary artery) who underwent mitral valve replacement (MVR) and coronary artery bypass grafting. She had previously undergone MVR and had been treated by the Takeuchi procedure for mitral valve regurgitation and a coronary artery anomaly, respectively, at 17 years of age. She was hospitalized with shortness of breath within 7 years of surgical intervention. Swan-Ganz catheterization revealed mild pulmonary hypertension and elevated mean pulmonary wedge pressure. Echocardiography revealed marked severe calcification of the bio valve and moderate mitral valve regurgitation. Coronary angiography revealed retrograde collateral blood flow from the right coronary artery to the left coronary artery, but intrapulmonary tunnel was not visualized by pulmonary arteriography. We performed MVR with a mechanical valve and coronary artery bypass grafting between the left internal thoracic artery and the left anterior descending artery. The postoperative course was unremarkable, and no complaints were reported during follow-up at the outpatient clinic.     

Direct aortic reimplantation for BWG syndrome including mitral valve repair

Direct aortic reimplantation is the ideal treatment for the BWG syndrome, but is often impossible to obtain a sufficient length of the coronary artery without undue tension on the vessel. We experienced a 6-year-old boy whose left coronary artery was originated from pulmonary trunk widely distant from aorta. Direct reimplantation was successfully performed taking care of excising the ostium of the anomalous vessel as a largest flange and mobilizing the coronary artery from the adjacent tissue sufficiently. Mitral valve regurgitation is also a poor prognosis factor of BWG syndrome. We performed direct reimplantation and mitral valve repair (anterior commissuroplasty) simultaneously to a 3-year-old boy of BWG syndrome with severe mitral valve regurgitation. Postoperative data showed improvement of cardiac performance. Possibility of recovering the papillary muscle function by valve repair without replacement was suggested.     

The hemodynamic effects and mechanism of action of pulmonary artery balloon counterpulsation in the treatment of right ventricular failure during left heart bypass

The efficacy of pulmonary artery balloon counterpulsation (PABC) was evaluated in improving right ventricular (RV) output during left heart bypass for global cardiac failure. In 13 pigs, a 40-ml balloon was positioned within a graft anastomosed to the pulmonary artery distal to the pulmonary valve, and left heart bypass was instituted from the left atrium to the carotid artery. Global myocardial failure was produced by an infusion of propranolol (range, 25 to 78 mg). In this model, RV output decreased despite volume loading to a right atrial pressure of 15 mm Hg and atrioventricular sequential pacing at 100 beats per minute. Pulmonary artery balloon counterpulsation increased both RV output (from 519 +/- 76 to 1,117 +/- 110 ml/min; p less than 0.01) and RV systolic stroke work (from 1.3 +/- 0.4 to 2.3 +/- 0.6 gm-m; p less than 0.01). Right atrial pressure decreased (from 15.5 +/- 0.9 to 10.7 +/- 1.0 mm Hg; p less than 0.01) in 8 of the pigs studied during RV failure. In 5 pigs, ventricular fibrillation occurred without a stable model of RV failure, and there was no cardiac output before or after counterpulsation. The mechanism of action of PABC was studied by placing a flow probe around a large branch of the right pulmonary artery. During RV failure, balloon inflation caused flow through the pulmonary circulation, and ventricular systole resulted in filling of the graft. During ventricular fibrillation, balloon inflation and deflation produced only a to-and-fro movement of blood in the pulmonary artery branch without net forward flow.(ABSTRACT TRUNCATED AT 250 WORDS)     

Left main coronary trunk compression by dilated main pulmonary artery in a patient with atrial septal defect

A 12-year-old girl with atrial septal defect combined with pulmonary hypertension and 90% stenosis of the left main coronary artery caused by dilated pulmonary artery was scheduled for atrial septal closure and coronary artery bypass graft under general anesthesia. During the echocardiographic examination to evaluate the anatomical relationship between the pulmonary artery and left main coronary trunk, bradycardia and a depression of ST-segment on electrocardiogram appeared suddenly when the operator compressed the pulmonary artery with a probe of echocardiography from the operative field. The circulatory collapse and ischemic change on electrocardiogram might have been caused by a further reduction of blood flow to the left main coronary trunk narrowed originally by dilated pulmonary artery. Although various etiologies, such as atherosclerosis, syphilis, and congenital abnormalities are widely known to cause stenosis of the left main coronary trunk, external compression by dilated pulmonary artery has not been widely known. Malignant arrhythmias from coronary artery compression with subsequent ischemia could contribute to an incidence of sudden death. Coronary angiography and magnetic resonance imaging are useful for the preoperative evaluation. Careful management is needed to protect such a patient from ischemic event in the perioperative period.     

Repair of an aneurysm of the pulmonary trunk in a 65-year-old patient

The case of a 65-year-old patient with asymptomaticaneurysm of the pulmonary trunk associated with severe insufficiency of the pulmonary valve and symptomatic coronary artery disease is presented. The surgical procedure included coronary artery bypass grafting, aneurysmectomy, and pulmonary artery replacement with implantation of a stentless bioprosthesis and lengthening of the root of the bioprosthesis with a reversed vascular Y prosthesis, which was anastomosed to the left and right pulmonary artery.