New surgical repair for an anomalous origin of the left coronary artery adjacent to the pulmonary cusps with numerous large side branches

A new surgical procedure for anatomical correction of an anomalous origin of the left main coronary artery (LCA) from the pulmonary artery is described for those patients in whom the origin of the LCA is located adjacent to a pulmonary cusp and several side branches are arising immediately behind the ostium of the LCA. Hence the LCA can not be disconnected from the pulmonary artery for either coronary arterial transfer or bypass grafting. A tunnel-type repair may interfere with the movement of the pulmonary leaflets. In those cases our proposed surgical correction consists of an arteriotomy of the pulmonary trunk, end-to-end anastomosis of a vein graft to the ostium of the LCA and its implantation into the ascending aorta. The resulting defect in the pulmonary artery is closed with a pericardial patch avoiding kinking or stenosis of the pulmonary trunk.     

Anomalous origin of the main stem of the left coronary artery from the pulmonary trunk presenting with left ventricular hypertrophy

Patients with anomalous origin of the left coronary artery from the pulmonary trunk may present with paroxysmal angina on exertion, congestive heart failure, dyspnoea, syncope or sudden death. The association of such anomalous origin of the left coronary artery from the pulmonary trunk with a hypertrophic left ventricle is extremely rare. In our cohort of patients with anomalous origin of the left coronary artery from the pulmonary trunk, two presented with a hypertrophic left ventricle. We discuss these cases, accompanied by a review of the English literature describing different morphological anomalies of the coronary arteries associated with left ventricular hypertrophy. Whether the hypertrophy is a result of the evolvement of the collateral coronary system, or due to an additional pathological gene for hypertrophic obstructive cardiomyopathy, remains an enigma. The hypertrophy may have served as a compensatory mechanism accounting for the atypical clinical presentation. We further discuss the possible factors associating the occurrence of anomalous origin of the left coronary artery from the pulmonary trunk and myohypertrophy, supported by the documentation of the regression of hypertrophy following surgical correction of the anomalous arterial origin as seen in our patients.     

Tl-201 perfusion scintigraphy of the myocardium in patients with anomalous origin of the left coronary artery from the pulmonary trunk (clinico-anatomical comparisons)

The clinical significance of 201Tl myocardial perfusion scintigraphy was studied in 7 patients with abnormal branch of the left coronary artery from the pulmonary trunk and in 1 patient with that of the anterior interventricular artery from the pulmonary trunk. Radiological criteria for the defect were established on the basis of comparative analysis of the data of myocardial scintigraphy with those of electrocardiographic, coronary angiography and anatomic examinations. It was shown that it was feasible to make a differential diagnosis between abnormal left coronary artery branch and dilated cardiomyopathy and that the technique was of value for comparative assessment of the efficacy of various types of surgical treatment for abnormal left coronary artery ramus from the pulmonary trunk.     

Surgical repair of the anomalous origin of the right pulmonary artery from the ascending aorta

The anomalous origin of the right pulmonary artery (AORPA) from the ascending aorta is a rare congenital malformation. We describe an infant who underwent a surgical correction with direct anastomosis between the right pulmonary artery and the pulmonary trunk. Eighteen months later, the patient remains asymptomatic, and no significant residual stenosis was detected on angioresonance.     

Multiple proximal coronaro-pulmonary fistulae. Review of the literature apropos of a new case

The authors report a new case of multiple proximal coronaro-pulmonary fistula between right coronary arteries, anterior interventricular artery and the trunk of the pulmonary artery, in a 64 year-old female patient with chest pain and a continuous murmur located in the third left intercostal space. The coronary steal is demonstrated by a myocardial scintigraphy during stress with return to normal after surgical ligation. A review of the literature enabled to find 33 cases of this major congenital anomaly of the coronary arteries, defined as an abnormal communication between at least two main coronary vessels and the trunk of the pulmonary artery. This results in a left-right shunt, usually minor without any repercussions on the right cavities and pulmonary pressures. The entire clinical, electrocardiographic, radiological, sonographic, scintigraphic, haemodynamic and angiographic picture is reported for these 33 cases. A physiopathological discussion is proposed. The course of this disease is usually favorable (only one case of myocardial infarction was published, without cardiac failure. Osler's endocarditis or sudden death); this seems to authorize simple monitoring as a logical therapeutic approach except when a myocardial ischemia secondary to coronary steal is demonstrated, imposing a surgical correction.     

Aberrant right coronary artery occlusion during the percutaneous pulmonary trunk stenting in a patient with tetralogy of Fallot

Aberrant coronary arteries are frequently observed in patients presenting with Fallot’s tetralogy (TOF). Before the complete surgical repair of the TOF, the percutaneously performed pulmonary trunk (PT) angioplasty is often performed in order to temporarily increase the pulmonary circulation, thus increasing the pulmonary vessel size, finally improving surgical outcome. This case reports a 12-year-old boy with a TOF insufficiently improved by surgical correction, in whom a PT angioplasty with stent implantation was complicated by an extrinsic compression of an aberrant right coronary artery (RCA) causing a myocardial ischemia with severe hypotension. The RCA, originating from the left anterior descending coronary artery, passed through the aortic root and the PT and was thus compressed by the PT-stent. Finally the RCA was successfully treated with standard coronary balloon angioplasty and stenting, improving myocardial perfusion and the hemodynamics of the patient, who finally died several days thereafter due to septic shock and massive pulmonary embolism.     

Anomalous origin of one pulmonary artery from the ascending aorta

We describe the surgical repair in three infants presenting with one pulmonary artery arising from the ascending aorta, the other artery arising normally from the right ventricle via the pulmonary trunk. Repair consisted of reimplantation of the anomalous pulmonary artery to the pulmonary trunk, in association with repair of associated intracardiac malformations. All patients survived the surgical procedures, and were discharged in stable clinical condition. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and underwent reoperation. Although survival after operation is now expected for this malformation, reports of late results are lacking. Larger numbers of operations are needed before we can reach definitive conclusions. The origin of one branch pulmonary artery from the ascending aorta in the presence of a pulmonary valve and main pulmonary artery is a very rare congenital cardiac anomaly. PATIENTS AND METHODS: Between January 1995 and June 2003, 3 infant girls presented with the origin of one branch artery from the ascending aorta, while the other pulmonary artery originated from the pulmonary trunk which was in continuity with the right ventricular outflow tract. The pulmonary artery that arose from the right ventricle was left in 2 and right in 1 patient. RESULTS: At the age 13, 48 and 62 days respectively, the patients underwent surgical repair consisting with reimplantation of the anomalous pulmonary artery branch to the pulmonary trunk in association with repair of intracardiac malformations. There were no hospital deaths. Postoperative complications included: prolonged intubation in two patients (10 and 16 days), low output syndrome in 1 patient, cardiac tamponade in 1 patient and seizures in 1 patient. All patients were discharged in good clinical condition. There have been no late deaths. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and after unsuccessful balloon dilation, underwent surgical reoperation. CONCLUSIONS: Although operative survival is now possible for this malformation, reports of late results are lacking. Two of the three patients developed stenosis at the surgical anastomosis relatively early after surgery. Larger numbers of operations are necessaries to reach definitive conclusions.     

Anomalous origin of the left coronary from the trunk of the pulmonary artery. Description of a clinical case

The case of a 32 year old woman with anomalous origin of the left coronary artery from the pulmonary trunk is reported, in whom, for technical difficulties, the surgical correction has been limited to artery ligation. After surgery, there was a complete clinical improvement and normalization of the stress electrocardiogram. Nevertheless, radioisotopic studies--exercise Thallium 201 scintigraphy and radionuclide ventriculography--disclosed a poor left ventricular function. It is possible to suppose that the prolonged perfusion deficit resulted in an extensive area of myocardial fibrosis.     

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.     

Anomalous origin of the left anterior descending coronary artery from the pulmonary trunk.

An extremely rare congenital abnormality is reported in which the anterior descending branch of the left main coronary artery arises independently from the pulmonary trunk in a young patient who presented with unstable angina. Its clinical presentation, angiographic identification and surgical treatment are described. It appears to be a distinct entity with few of the features of the classical form of anomalous origin of the left main coronary artery.     

Anomalous left coronary artery originating from the pulmonary artery. Report on 15 cases.

Fifteen infants and children with the diagnosis of anomalous left coronary artery from the pulmonary trunk have been encountered at the Children's Hospital Medical Center, Boston, Massachusetts from 1958 to 1973. After thorough clinical and laboratory evaluation, they have been treated by anticongestive measures. Nine patients have had ligation of the anomalous left coronary artery at its entrance into the pulmonary artery; one patient has undergone coronary bypass surgery. The lelctrocardiogram proved to be the most helpful diagnostic clinical laboratory test, Vectorcardiograms are valuable not only in diagnosis but also in the follow-up of the patients from the prognostic point of view. The most sensitive tool for the definitive diagnosis is an aortic rool angiogram; we have no false negatives or false positives with this method. The twelve patients with complete cardiac catheterization data could be divided into three groups, according to the pressure and magnitude of the left-to-right shunt at the pulmonary level. All patients with an appreciable le?T-TO-RIGHT SHUNT SURVIVED. Patients in whom no left-to-right shunt could be demonstrated by angiography died. Half of the patients with only small left-to-right shunt survived; The results of surgical and medical treatment, were identical within the three groups. Medical management in infancy, according to coronary care principles, with definitive surgical correction at a later age, is the preferred treatment. Ligation of the anomalous left coronary artery is recommended in severely symptomatic infants with documented left-to-right shunt at the pulmonary artery level, who do not respond to medical management.     

Massive pulmonary embolism associated with a right ventricular myxoma

A 12 year old boy had a massive pulmonary embolism associated with a right ventricular myxoma. This caused complete occlusion of the main trunk of the left pulmonary artery and of a branch of the right pulmonary artery supplying the basal area of the lower lobe of the right lung. The patient died despite two surgical attempts to remove the tumor clots. To our knowledge this constitutes the first report of a massive pulmonary artery embolism associated with a right ventricular myxoma.     

Surgical repair of naturally palliated anomalous origin of the left coronary artery from the right pulmonary artery.

Anomalous origin of the left coronary artery from the pulmonary arteries is a rare and life-threatening defect, usually needing prompt surgical correction during infancy. We describe the case of a young asymptomatic patient with this defect who underwent surgical reimplantation despite the absence of signs of myocardial ischemia, due to the presence of proximal stenosis of the anomalous coronary artery.     

Ectopic and atretic ostium of left main coronary artery: from interleaflet triangle between left and noncoronary cusps

Left main coronary artery atresia is an extremely rare coronary anomaly in which there is no left coronary ostium and the left main trunk ends blindly. Ectopic origin of the left coronary artery from the left ventricle is an even rarer condition. Herein, we describe the case of a 37-year-old woman whose left main coronary artery atresia was not corrected during previous surgical correction of patent ductus arteriosus and aortic insufficiency. Five years after that first operation, the patient developed severe angina and heart failure. She underwent coronary artery bypass grafting with greater saphenous vein directly to the left main coronary artery trunk, along with associated procedures. The operative findings revealed an ectopic and atretic ostium of the left main coronary artery from the interleaflet triangle between the left and noncoronary cusps, which we believe is a specific congenital coronary anomaly.     

Contribution of two-dimensional echocardiography to the diagnosis of abnormalities of the left coronary artery originating in the trunk of the pulmonary artery

The abnormal origin of the left coronary artery from the trunk of the pulmonary artery is rarely encountered, difficult to diagnose and has a severe prognosis because of the major risk of sudden death. The authors report the case of a 63 year-old male patient, presenting this anomaly which creates a true left-to-right shunt with myocardial ischemia secondary to the steal of coronary blood toward the pulmonary artery. The diagnosis is evoked by bidimensional cardiac sonography, pulsated and color Doppler, which permit to confirm the left-to-right shunt while this is confirmed by coronary angiography. The treatment is essentially surgical.     

Lobar emphysema due to anomalous aortic origin of the left pulmonary artery.

The unusual case of an infant with aortic origin of the left pulmonary artery is presented. The patient developed a rare complication of lobar emphysema due to bronchial compression from the enlarged right pulmonary artery. Operative anastomosis of the left pulmonary artery to the pulmonary trunk was successful, with subsequent resolution of the lobar emphysema.     

Primary stenting of the left main coronary artery with anomalous origin from the right sinus of valsalva

The presence of an aberrant origin of left main coronary artery from the right sinus of valsalva passing between the aorta ascendens and pulmonary trunk is one of the anomalies most frequently associated with malignant clinical events, and surgical treatment is recommended. We report of a 64-year-old patient with a highly increased risk for surgery due to severe chronic obstructive pulmonary disease. This case demonstrates that the use of a drug-eluting stent offers a suitable and valuable alternative.     

Anomalous origin of left pulmonary artery branch from the aorta with Fallot's tetralogy

We report the case of a ten-year-old boy who had often presented with respiratory distress since birth. Chest X-ray showed an enlarged right ventricle. Echocardiography demonstrated TGE characteristic features of Fallot's tetralogy and the left pulmonary artery could not be visualized. However, cardiac catheterization disclosed that the left pulmonary artery had an anomalous origin from the ascending aorta. The patient underwent total surgical correction of the defects. Nine days later, he was discharged.We present the results of a literature review of the incidence,physiopathological, clinical, diagnostic, and surgical characteristics of this rare disease.     

Simultaneous mesenteric and renal bypass for visceral ischemia and renovascular hypertension

Severe visceral ischemia and uncontrollable hypertension in a patient with previous aortofemoral and right aortorenal bypasses underwent surgical correction for stenosis of the celiac, superior mesenteric and left renal arteries. A Dacron jump graft from the junction of the aorto-celiac trunk to the superior mesenteric artery artery alleviated the mesenteric ischemia. A nonreversed vein graft connected the side of the Dacron prosthesis to the end of the left renal artery improved the renovascular hypertension. This modified, simple surgical approach, successfully corrected visceral ischemia and renovascular hypertension and avoided using the severely calcified aorta for celiac and mesenteric bypasses.     

Emergency trans‐catheter coronary intervention for left main compression secondary to pulmonary hypertension in a 4‐year‐old child

Here, we report on the case of a 4‐year‐old child with large atrial septal defect (ASD) and severe pulmonary hypertension presenting an ischemic cardiomyopathy secondary to left main (LM) compression by a dilated pulmonary artery trunk. Despite of surgical treatment consisting in ASD closure and coronary artery bypass grafting, the patient was not weanable from ECMO. Control coronarography showed a near‐occlusion of the left mammary bypass. A rescue percutaneous LM angioplasty with drug‐eluting stent implantation was performed.