Hemoptysis in a patient with acquired anomaly of the arterial vascularization of the lung

An anomalous systemic pulmonary supply may be acquired and present only one symptom: hemoptysis. The incidence of this pathology is unknown. A case of acquired systemic pulmonary arterialization was observed after surgical intervention of myocardium vascularization with the left mammary artery. The diagnosis was obtained through an arteriography of the left mammary artery. In this case, the therapy consists in ablating the affected parenchyma followed by the interposition of a bovine pericardium patch between the mammary artery and the remaining pulmonary tissue.     

Resuscitation in ventricular fibrillation as the first manifestation of Bland-White-Garland syndrome in adulthood

CASE REPORT: This case presents a 31-year-old male patient with anomalous origin of the left coronary artery from the pulmonary trunc. First symptom of the disease was a survived sudden cardiac death. Subsequent angiographic and echocardiographic studies demonstrated the anomalous origin of the left coronary artery from the pulmonary artery. There were no signs of prior myocardial infarction. After reimplantation of the anomalous originating left coronary artery no myocardial ischemia could be detected in the thallium-201 myocardial imaging, which was present before surgical correction. In this case myocardial ischemia was the only potential triggering mechanism responsible for the sudden cardiac death, which was no longer detectable after surgical correction. Therefore no additional pharmacological and nonpharmacological antiarrhythmic treatment was initiated. CONCLUSION: In rare cases the first manifestation of Bland White Garland syndrome in the adult patient could be sudden cardiac death due to ventricular fibrillation.     

Three unusual complications resulting from attempted repair of partial anomalous pulmonary venous drainage

The correction of shunts resulting from partial anomalous pulmonary venous drainage has become an accepted surgical procedure. Surgical complications, other than those that were purely postoperative, have been rare. The present report details the case histories of three patients with unusual complications resulting from this type of surgery. Unilateral pulmonary venous obstruction and repeated infections occurred in one patient. In another, obstruction of the superior vena cava resulted. In the third patient, an indaequate operation was performed when the site of partial anomalous pulmonary venous drainage into the coronary sinus was not recognized initially at the time of surgery.     

Anomalous double blood supply to the lung

We report an unusual case in which an apparently normal upper lobe of the right lung was supplied by major systemic arterial and pulmonary arterial vessels. The anomalous artery arose from the descending aorta. Following interruption of this vessel, the machinery-like murmur previously present disappeared.     

Bidirectional cavopulmonary shunt in patients with anomalies of systemic and pulmonary venous drainage

BACKGROUND: Bidirectional cavopulmonary shunt and Fontan repair are now commonly performed in patients with a variety of forms of complex single ventricle, including those with anomalies of systemic or pulmonary venous return. These anomalies are ideally dealt with during bidirectional cavopulmonary shunt, thereby minimizing the complexity of the eventual Fontan procedure. METHODS: Between March 1990 and December 1995, 36 patients with anomalous systemic or pulmonary venous drainage underwent bidirectional cavopulmonary shunt. A combination of anomalous systemic and pulmonary venous drainage was present in 12 patients, whereas 19 patients had anomalous drainage only from the systemic circulation and 5 patients had isolated anomalies of pulmonary venous return. Visceral heterotaxy syndrome was diagnosed in 18 patients. The median age at operation was 11 months, and bidirectional cavopulmonary shunt was the first surgical procedure performed in 10 of these patients. Techniques of repair are described. RESULTS: There were two early deaths and one bidirectional cavopulmonary shunt was taken down, for mortality and failure rates not significantly different than those for all patients undergoing bidirectional cavopulmonary shunt during this time period (n = 117). At a mean follow-up of 19.9 months, there have been three late deaths and 11 patients have undergone Fontan completion. Actuarial survival was 87% at 1 year and 81% at 3 years. Among all patients undergoing bidirectional cavopulmonary shunt during this time period, neither heterotaxy syndrome nor anomalies of systemic or pulmonary venous return were significantly associated with decreased survival or poor outcome. CONCLUSIONS: Bidirectional cavopulmonary shunt can be performed in patients with anomalous systemic or pulmonary venous drainage, including those with visceral heterotaxy syndrome, with morbidity and mortality rates that do not differ significantly from those achieved in all patients undergoing bidirectional cavopulmonary shunt. In this report, we describe our experience with this group of patients, primarily focusing on outcomes and technical issues that pertain to the use of bidirectional cavopulmonary shunt as a preparatory procedure for the extracardiac conduit Fontan operation.     

Scimitar syndrome in infancy

OBJECTIVES: The objectives of this study were to determine the anatomic and physiological factors most responsible for the severe symptoms and poor prognosis of infants with scimitar syndrome. BACKGROUND: Whereas the diagnosis of scimitar syndrome is often made incidentally in older children and adults who undergo chest radiography for diverse reasons, infants in whom the diagnosis is made typically present with severe symptoms and have a poor prognosis. METHODS: The clinical, catheterization and imaging data of 13 consecutive infants with scimitar syndrome who underwent cardiac catheterization in the 1st 6 months of life were reviewed, with emphasis on the pulmonary artery pressure, pulmonary and cardiovascular anatomy, therapeutic interventions and outcome. RESULTS: Twelve of the 13 infants had pulmonary hypertension at the time of diagnosis. Six patients died despite specific treatment. Eleven of 13 infants had associated cardiac malformations and 9 had large systemic arterial collateral channels to the right lung. Seven patients had anomalies involving the left side of the heart, especially varying degrees of hypoplasia of the left heart or aorta, and six of these patients died. Ten patients underwent surgical or transcatheter therapy in the 1st year of life. Systemic arteries to the right lung were ligated in three patients and occluded by transcatheter embolization in four. Balloon angioplasty was carried out in two patients, one with stenosis of the left-sided pulmonary veins and one with stenosis of the anomalous right pulmonary vein. The latter had placement of a balloon-expandable stent. In both patients, pulmonary vein stenosis progressed. Six patients had surgical repair of associated cardiovascular anomalies, and two required repair of extracardiac congenital anomalies. Occlusion of the anomalous systemic arteries was generally associated with clinical improvement, but congestive heart failure and pulmonary hypertension recurred in those patients with associated cardiovascular anomalies, whose condition subsequently responded after correction of the shunt lesions. CONCLUSIONS: The severe symptoms and pulmonary hypertension found in infants with scimitar syndrome have many causes. Anomalous systemic arterial supply, pulmonary vein stenosis and associated cardiovascular anomalies play a significant role, and the ultimate outcome of individual infants depends on the feasibility of treating these anomalies in early infancy.     

The superior approach for correction of the supracardiac type of total anomalous pulmonary venous return

An alternative approach for correction of supracardiac (type I) total anomalous pulmonary venous return is described. A median sternotomy is used. The posterior wall of the left atrium and the common pulmonary venous trunk are exposed through the transverse sinus. A direct anastomosis between these structures, ligation of the systemic-venous connection (vertical vein), and closure of the interatrial septal defect results in a one-stage repair. In our experience with the supracardiac anomaly in 20 patients, we have found that this approach consistently affords better exposure than other techniques currently in use for surgical correction of this anomaly.     

Horseshoe lung: demonstration by electron-beam CT

Horseshoe lung is a rare pulmonary anomaly characterized by fusion of the posterobasal portions of the right and left lungs behind the pericardial reflection, anterior to the aorta. The majority of reported cases occur in conjunction with scimitar syndrome, including hypoplasia of the right lung, anomalous right pulmonary venous return and systemic arterial supply to the lung. Horseshoe lung is usually diagnosed on pulmonary arteriography when the right inferior pulmonary artery crosses the midline and extends to the left lung base. Bronchography is also diagnostic when the branch of horseshoe portion arises from the right bronchus and passes within the lung parenchyma to midline of the lung tissue. The only described CT finding of horseshoe lung is the contiguity of the right and left lungs behind the heart. Most cases are infants under 12 months of age and CT images are severely hampered by respiration motion artefacts. Such artefacts are minimized by using electron-beam computed tomography, allowing a more detailed CT appearance of horseshoe lung in this case.     

Surgical management of tricuspid atresia and anomalous left brachiocephalic vein

An anomalous left brachiocephalic vein is an uncommon systemic venous anomaly, which usually has no clinical significance. We describe a case of tricuspid atresia with such an anomalous left brachiocephalic vein. The presence of this unusual venous anomaly had a number of implications in the surgical management of the tricuspid atresia.     

Left partial anomalous pulmonary venous connection found during a lobectomy for lung cancer: report of a case

We report herein the case of a 68-year-old man in whom a partial anomalous pulmonary venous connection (PAPVC) was found during an operation for primary lung cancer. The preoperative clinical findings did not suggest a vascular shunt, and intraoperatively the anomalous vein was seen to drain only from the left upper lobe into the left innominate vein. The lower pulmonary vein connected normally, and there was no atrial septal defect nor any other anomalous condition. A left upper lobectomy with ligation of the anomalous connected vein was performed uneventfully. This type of PAPVC is extremely rare, and is especially noteworthy because there were no clinical signs.     

Is vertical vein ligation necessary in repair of total anomalous pulmonary venous connection?

BACKGROUND: In the repair of total anomalous venous connection, vertical vein ligation is recommended to eliminate left-to-right shunting. However, the small left heart chambers may not always tolerate the immediate increase in blood flow after combined repair and vein ligation. METHODS: A retrospective review of 23 infants and children undergoing correction of total anomalous pulmonary venous connection was undertaken to determine whether vertical vein ligation is a necessary component of successful surgical repair. In 14 patients this vein was ligated, whereas in 9 it was left patent. Six patients who underwent ligation and 5 who did not had pulmonary venous obstruction before operation. RESULTS: The operative mortality rate was 36% (5 of 14 patients) for the ligated group compared with 0% (0 of 9 patients) for the nonligated group (p = 0.06). All deaths occurred in patients with preoperative obstruction and a low mean left atrial pressure, and four of the deaths were directly attributable to left heart failure. Follow-up echocardiography in patients in whom the vertical vein was not ligated revealed adequate cardiac function and no residual left-to-right flow through the previously patent venous conduit. CONCLUSION: Vertical vein ligation during the repair of total anomalous pulmonary venous connection is not routinely necessary and actually may be undesirable in patients with preoperative obstruction, in whom the left heart chambers are particularly small.     

Pulmonary regurgitation in large atrial shunts without pulmonary hypertension

Seven patients with pulmonary regurgitation (PR), normal pulmonary artery (PA) pressures and large left-to-right atrial shunts are reported. Six had secundum atrial septal defects (ASD) and one had anomalous pulmonary venous drainage. These comprised 4% of 180 patients with atrial shunts and normal PA pressures. Pulmonary regurgitation was diagnosed clinically by mid-frequency diastolic decrescendo murmurs beginning after the pulmonic component of the second heart sound, and diagnoses were confirmed by catheterization. In two patients who had serial preoperative catheterizations over 8 and 16 years, PR progressed in one and was present only on the second study in the other. All patients underwent shunt correction, at which time the pulmonic anulus and artery appeared dilated, but the pulmonic valves were normal and did not require revision. In all patients the PR murmur disappeared after shunt correction alone, and on chest X-ray both PA and overall heart size decreased. Although it is known that pulmonary regurgitation occurs with atrial septal defects and pulmonary hypertension, the present study demonstrates that it also occurs with high flow atrial shunts, in which setting it has different implications and is reversible with shunt correction alone.     

Treatment of local recurrence of rectal cancer

We report on 2 infants with Ullrich-Turner syndrome in whom partial anomalous pulmonary venous drainage was diagnosed noninvasively by color-coded Doppler sonography. Several patients with the combination of anomalous drainage of one or more pulmonary veins and Ullrich-Turner syndrome have been described in the literature. However, in the majority of those previously reported cases the diagnosis of partial anomalous pulmonary venous drainage was established by angiography during cardiac catheterization performed for confirmation of other cardiovascular malformations. Our patients show that partial anomalous pulmonary venous drainage can be diagnosed easily in neonates and young infants, as long as this anomaly is taken into consideration.     

The operability of patient with pulmonary hypertension judged from hemodynamic changes before and after surgery

1) In congenital heart disease with pulmonary hypertension, the place for surgical correction still remains even with almost systemic PA pressure if R to L shunt ratio on lung scintigram is lower than 25%. 2) The patient with pulmonary hypertension in congenital heart disease younger than 3 years of age has wider acceptability for surgery compared to older patient. 3) In patients with mitral valvular disease, surgical correction seems to be indicated irrespective of PA pressure, if their preoperative U/L are lower than 2.4.     

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.     

Bilateral ductal origin of the pulmonary arteries. Systemic-pulmonary arterial anastomosis as first stage in planned total correction

Absence of continuity between the heart and the pulmonary arteries has the potential for total correction. When the pulmonary arteries are narrow, a palliative first stage systemic-pulmonary arterial anastomosis serves to enlarge the pulmonary arteries so that, with time, they become of adequate caliber for total correction. In a case with bilateral ductal origin of pulmonary arteries, palliative anastomosis between the aorta and right pulmonary artery was performed as the first stage in a program planned for ultimate total correction.     

Incidence and spectrum of spontaneous neoplasms in Han:NMRI mice of both sexes

Pulsed Doppler ultrasound has been used to characterize distinctive pulmonary venous flow patterns in the normal fetus and child. Changes in these characteristic flow patterns have been related to abnormal atrial and ventricular hemodynamics. We report a case of total anomalous pulmonary venous return diagnosed prenatally because of an abnormal pulsed Doppler echocardiographic flow pattern, even though color flow mapping appeared to demonstrate normal pulmonary venous drainage. This case demonstrates the importance of obtaining pulsed Doppler pulmonary venous flow profiles during fetal echocardiography, especially in cases of complex congenital heart disease.     

Pryce type I interlobar pulmonary sequestration with anomalous return to the left inferior pulmonary vein

A 50-year-old woman was examined because of chest discomfort. Chest X-ray films disclosed a tumorous shadow behind the heart. Chest computed tomography (CT) scans revealed a mass connected to the descending aorta, with increased blood flow in the left basal segment. The patient was admitted for further examination. Chest CT scans and cardioscintigrams were very useful as diagnostic tools, but the final diagnosis was made on the basis of angiography. An anomalous tortuous artery ran from the descending aorta into the left basal segment and returned to the left inferior pulmonary vein. The left pulmonary arterial trunk had no basal branch (A8-A10). A loop corresponding to the superior vein (V6) ran beneath the anomalous tortuous artery. To our knowledge, this is the second case of Pryce type-I interlobar pulmonary sequestration with anomalous return to the left inferior pulmonary vein to be reported in Japan. A left inferior lobectomy was performed. Histological finding from the excised tissues showed prominent interstitial fibrosis, atypical adenomatous hyperplasia, and atherosclerosis. Following surgery, the patient's PaO2 increased from 80.4 Torr to 95.8 Torr, suggesting that left inferior lobectomy was an appropriate treatment.     

Hemodynamics in sepsis: its relationship to pulmonary complication

Serial clinical observations and hemodynamic measurements were made on seventeen patients with sepsis and ten control cases. Control patients were slightly hypovolemic immediately after operation. In the eleven high flow patients, that is, those with the initial cardiac index higher than 3.0 liters/min/m(2), we observed characteristic hemodynamics with lowered vascular resistance, higher cardiac output and hypotension. The anomalous vascular resistance began to return to normal approximately 48 hr after operation. In three cases pulmonary complication developed at this time and therefore pulmonary artery pressure and/or pulmonary wedge pressure exceeded the normal range. In these cases treatment was aimed at lowering the pulmonary pressure. Pulmonary complication was improved when these pressures returned within normal range. In the seven low flow patients, that is, those with cardiac index lower than 2.9 liters/min/m(2), we observed decreased cardiac index with increased systemic and pulmonary vascular resistance. In these cases pulmonary complication appeared to develop by increased permeability of the pulmonary capillary wall. Elevated pulmonary artery pressure might accelerate the progress of the complication. Monitoring and controlling pulmonary artery pressure and pulmonary wedge pressure were effective in prevention and treatment of pulmonary complication in this group.     

Handedness assessment inventory

A 6 year old boy with a large atrial septal defect, partial anomalous pulmonary venous drainage and unrecognized anomalous insertion of the inferior vena cava into the left atrium had cyanosis after closure of the atrial defect. Repeat study revealed direct drainage of the inferior vena cava into the left atrium with moderate arterial oxygen desaturation. At repeat operation an unusual positioning of the inferior vena cava was seen. After reopening of the atrial defect, the pulmonary venous and systemic venous drainage anomalies were identified. A Dacron patch was inserted so as to divert flow to the proper atrium. Repeat catheterization 3 months after operation revealed a normal heart with no obstruction; arterial oxygen saturation was normal. The child has continued to do well 3 years after operation.