Differential pulmonary flow in hypoplastic left heart syndrome.

We report a case of hypoplastic left heart syndrome associated with restrictive interatrial communication and partial anomalous pulmonary venous connection via a right lower pulmonary vein draining to the inferior vena cava. We found unequal pulmonary artery pressure and different pulmonary artery structure, with the right pulmonary artery being lower in pressure and more tortuous and dilated in its peripheral branches than the left. This was attributed to the variant degrees of pulmonary venous obstruction. The left pulmonary venous return was severely obstructed by the restrictive interatrial communication, whereas the anomalous right lower pulmonary vein drained into the inferior vena cava, with less obstruction. To the best of our knowledge, there has never before been a report of differential pulmonary flow associated with a partial anomalous pulmonary vein, in a case of hypoplastic left heart syndrome.     

Systemic arterial supply to the normal basal segments of the left lower lobe: radiographic and CT findings in 11 patients

SUMMARY: To assess the radiologic findings of systemic arterial supply to the normal basal segments of the left lower lobe, chest radiographs and CT scans of all 11 patients were reviewed retrospectively by two chest radiologists. On posteroanterior chest radiographs (n = 11), the anomalous systemic artery arising from the descending thoracic aorta was shown as a retrocardiac mass (n = 11). Absence of normal lower lobar pulmonary artery shadow (n = 11), partial obliteration of the descending aortic interface (n = 9), and increased interstitial markings in left lower lung zone (n = 8) were also noted. On contrast-enhanced scans (n = 11), abrupt tapering of the normal left lower lobar pulmonary artery distal to the origin of superior segmental artery (n = 11), aberrant systemic artery originating from the descending aorta (n = 11), mild volume loss in the left lower lobe (n = 11), diffuse dilatation of the intrapulmonary peripheral vasculature (n = 11) and areas of ground-glass opacity (n = 10) in the basal segments of the left lower lobe were seen. Because chest radiography and CT consistently demonstrate several combinations of characteristic findings, conventional aortography may not be necessary for proper diagnosis of this anomaly.     

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.     

Two cases of intralobar pulmonary sequestration associated with nontuberculous mycobacterial infection in a young patient]

We report two cases of intralobar pulmonary sequestration associated with nontuberculous mycobacterial infection. Case 1: A 28-year-old woman was hospitalized because of cough. Chest CT demonstrated multi-cystic lesions with an air-fluid level in the right lower lobe. Case 2: A 25-year-old woman was hospitalized because of fever. Chest CT demonstrated homogeneous consolidation in the right lower lobe. In both cases, aortography showed an anomalous artery supplying the right basal segment, and pulmonary sequestration was diagnosed. Furthermore, Mycobacterium avium was detected in the bronchial lavage, and a right lower lung lobectomy was performed after chemotherapy. Mycobacterium avium was cultured from the fluid in the sequestrated lung, and histological findings of the resected lobe showed epithelioid cell granulomas. We diagnosed these cases as having pulmonary sequestrations associated with nontuberculous mycobacterial infection. We concluded that pulmonary sequestration should be considered an underlying disease of secondary nontuberculous mycobacterial infections in young patients.     

Pulmonary artery sling as a cause of recurrent wheezing in children

Vascular rings are a group of aortic arch anomalies and usually presents with dispnea, stridor and feeding difficulties after birth. Pulmonary artery sling is created by anomalous origin of the left pulmonary artery from the posterior aspect of the right pulmonary artery. The anomalous left pulmonary artery compresses the lower trachea and right mainstem bronchus, producing upper airway symptoms. We presented a seven-month-old male patient who admitted to our clinic because of recurrent wheezing, stridor, and frequent lower respiratory tract infections.     

Anomalous systemic arterial supply to normal basilar segments of the lower lobe of the left lung.

A 38-year-old man with an anomalous systemic arterial supply from the descending thoracic aorta to the normal basilar segments of the left lower lobe experienced symptoms of progressive exertional dyspnea. Although the pulmonary parenchyma was normal, there was no pulmonary arterial supply to the basilar segments of the left lower lobe. Left lower lobectomy was performed because of worsening left-to-left shunt. Aortography and pulmonary arteriography form the cornerstone for diagnosis, but a less invasive diagnostic method with less patient suffering is high-resolution computed tomography, which might replace bronchography for accurate evaluation of the distribution and patency of the bronchial tree and delineation of the characteristic "Medusa's hair"-like anomalous vascular supply penetrating into the nearly normal pulmonary parenchyma.     

Primary congenital anomalies of the coronary arteries: a coronary: arteriographic study

Geographic variations in the incidence of different congenital coronary anomalies are well known, but infrequently studied in the Indian population. Among 4,100 adult patients who underwent diagnostic coronary arteriography, 39 (0.95%) patients (34 males, 5 females) had one or more anomalous coronary arteries. Their mean age was 46.4 +/- 8.2 years (range, 26-68 years). Thirty-five (89.74%) had anomalies of origin and distribution, while the remaining four (10.25%) had coronary artery fistulae. Right coronary artery was the commonest anomalous vessel, involved in 19 (48.74%) patients. It was originating from the left sinus of Valsalva in 15 and from the non-facing aortic sinus in four patients. Anomalous left circumflex artery was the second commonest anomaly, seen in 14 (35.89%) patients. Anomalous left anterior descending artery and anomalous left coronary artery from pulmonary artery were seen in one patient each. Among patients with coronary fistulae, two had fistulae between the left anterior descending artery and the main pulmonary artery, one between the conal artery and the right atrium, while the fourth patient had fistulae from the right coronary as well as from the left anterior descending artery to the left atrium. Atherosclerotic plaques in the anomalous arteries were seen in only 13 (33.33%) patients, much less than the overall incidence of coronary artery disease in patients with congenital coronary anomalies in this series (66.66%). In four (10.25%) patients, only the anomalous vessels were involved in coronary artery disease. Thus, in a small subgroup there does not appear to be an increased risk for development of atherosclerotic coronary artery disease in anomalous coronary arteries. Recognition of coronary anomalies is important in patients undergoing coronary arteriography, coronary interventions and cardiac surgery. Variations in the frequency of primary congenital coronary anomalies may possibly have a genetic background.     

Occlusion of an aberrant artery to a pulmonary sequestration using a duct occluder

This report describes a female infant with a rare chromosome defect, del. 12 (q22-24.1), who has severe pulmonary valve stenosis, an atrial septal defect, and a small muscular ventricular septal defect. At 4 months of age a balloon pulmonary valvuloplasty was performed in the cardiac catheterization laboratory. During the procedure, a large aberrant artery from the aorta to a sequestration of the right lower lobe of lung was found. The flow-off from the sequestration was into a dilated left atrium. The single artery supplying the sequestration was successfully occluded using an Amplatzer Duct Occluder device. There were no complications and the infant remains well at 1-year follow-up.     

Broken-heart syndrome (Takotsubo syndrome)

We report on the rare case of partial anomalous return of four pulmonary veins in the right atrium and superior vena cava with intact interatrial septum in a five-year-old child. There were few symptoms in contrast with the left ventricular output dependent on the flow of the left upper lobe vein and from the lingula. Reduced compliance to the left led to a severe picture of pulmonary venocapillary hypertension in the immediate postoperative period, mitigated by an 8-mm interatrial septal defect. The patient progressed well after the intervention.     

Pulmonary arterial stenosis with wide splitting of the second heart sound due to mediastinal fibrosis

Mediastinal fibrosis led to complete obstruction of the left main pulmonary artery and partial stenosis of the pulmonary artery to the right lower lobe in a 48 year old man. The lesion resulted in a high pressure pulmonary vascular system with an area of prolonged runoff through a low pressure vascular bed. Physical findings included a systolic murmur that radiated to the right hemithorax and a widely split second heart sound. The wide splitting was attributed to delay in deceleration of the column of blood due to partial stenosis of the pulmonary artery to the right lower lobe.     

Intralobar sequestration of the lung presenting as a continuous murmur--a case report.

An eighteen-year-old asymptomatic woman with a continuous murmur, normal two-dimensional echocardiogram, and mild haziness of the left lower segment of the lung on chest x-ray was diagnosed to have sequestration based on the presence of a large anomalous systemic artery arising from the descending thoracic aorta supplying the lower lobe of the left lung. Normal bronchial connections with the rest of the lung on bronchoscopy and delayed dye clearance from the left lower lobe bronchioles on bronchogram indicated its intralobar nature. The cause of the continuous murmur was thought to be the large flow through the anomalous systemic artery.     

Partial anomalous pulmonary venous drainage and intact atrial septum with mitral stenosis: the paradox of a small shunt

Clinical, hemodynamic, and angiographic features of 10 patients with partial anomalous pulmonary venous drainage, intact atrial septum, and rheumatic mitral stenosis have been presented. Seventeen patients with this combination of anomalies reported in the literature have also been reviewed. The clinical diagnosis of mitral stenosis was possible in each of our 10 cases. Partial anomalous pulmonary venous drainage and intact atrial septum in addition to mitral stenosis was clinically suspected in only four patients. The findings suggesting additional presence of partial anomalous pulmonary venous drainage and intact atrial septum in a patient with mitral stenosis include: wide variable splitting of the second sound, pulmonary ejection systolic murmur with or without a thrill, and radiological evidence of unilateral increase in pulmonary vascularity or unilateral hilar pulsations. Hemodynamic findings were characterized by a relatively modest left to right shunt (Qp/Qs:2.2 +/- 1.4). Elevated pulmonary vascular resistance was found in the anomalously draining as well as the normally draining lung segments (9.1 +/- 4.9 and 6.5 +/- 3.4 units, respectively, t = 1.32;NS). The mechanism of the unexpected high resistance in the anomalously draining lung segments permitting only a small left to right shunt is discussed.     

Unusual vascular anomalies causing persistent pulmonary hypertension in a newborn.

A unique case of pulmonary vascular anomalies causing persistent pulmonary hypertension in a newborn is described. The child died 3 days after birth. Necropsy revealed marked hypoplasia of the right and left pulmonary arteries with a normal main pulmonary artery, patent ductus arteriosus, bilateral systemic arteries to the lungs from the abdominal aorta, and partial anomalous pulmonary venous connection. Quantitative morphometric techniques demonstrated slight abnormalities of alveolar development and severe arterial medial hypertrophy with abnormal extension of muscle into small peripheral arteries. Bronchopulmonary development appeared relatively normal in spite of the vascular abnormalities.     

Fetal Diagnosis of Abnormal Origin of the Left Pulmonary Artery

Isolated anomalies of the branch pulmonary arteries are rare, more often occurring in the setting of complex congenital heart disease. These isolated anomalies are often not identified in the prenatal period. We describe two cases of isolated anomalies of the left pulmonary artery which were identified on fetal echocardiography and confirmed postnatally, an anomalous left pulmonary artery arising from the base of the left‐sided brachiocephalic artery in the setting of a right‐sided aortic arch, and a left pulmonary artery sling. These two cases support our current understanding of normal and abnormal development of the extrapericardial arterial vessels and highlight the importance of meticulous attention when sweeping from the three‐vessel tracheal view.     

Bilateral superior vena cava with right superior vena cava draining into left atrium

Anomalies of systemic venous return are extremely heterogeneous congenital malformations with variable ranges from completely normal physiology to severe forms of right to left shunting requiring surgical treatment. Anomalous drainage of a right-sided superior vena cava (SVC) to the left atrium (LA) is one of the rarest variants of systemic venous return anomalies, characterized by right-to-left shunt physiology and cyanosis. Here we report a 2 years old girl presented with cyanosis which was observed shortly after birth by her parents but not further investigated. She is otherwise active girl and with normal growth and development. Her clinical examination was unremarkable apart from mild clubbing of the fingers and low oxygen saturation of 88–90% in room air. Her ECG and chest X-ray were unremarkable. Echocardiography showed bilateral SVC connected by a small innominate vein. The right SVC drains directly into the LA while the left SVC drains into the right atrium (RA) via a dilated coronary sinus. There is a small superior sinus venosus type atrial septum defect (ASD) with left to right shunt. Also, there is partial anomalous pulmonary venous return with right upper and right middle pulmonary veins draining directly into the right SVC, which is connected to LA. The right lower pulmonary vein and left pulmonary veins drain directly to LA. The rest of her echocardiography demonstrated normal heart structures and function. This patient was referred for surgical correction, including baffling of the right SVC to the RA and closure of the ASD. We describe this case to highlight the importance of recognizing this rare anomalous systemic venous connection as one of the very rare causes of cyanosis in the pediatric age group as well as at older age.     

冠状动脉起源异常的检出率与冠状动脉狭窄

目的　评价国人冠状动脉(冠脉)起源异常的冠脉造影检出率,并探讨其与冠脉狭窄之间的关系。方法　回顾性分析2001年1月至2004年6月在我院接受选择性冠脉造影的患者,观察并记录冠脉起源异常的检出频度和类型,以及合并冠脉狭窄的情况。结果　4094例患者中,共检出冠脉起源异常者32例,检出率为0 78%,其中右冠脉异常起源最为多见,占总数的65 6%;合并冠脉狭窄者共11例(占34 3% ), 其中仅5例(占15 6% )累及起源异常的冠脉,且无1例冠脉狭窄单独累及起源异常的冠脉。结论　国人冠脉起源异常的检出率与国外文献报道结果相似,其中以右冠脉起源异常最为多见,冠脉起源异常与冠脉狭窄不具有相关性。     

Anomalous origin and course of the coronary arteries

In the normal heart, the right and left coronary arteries arise from the aortic valvar sinuses adjacent to the pulmonary trunk. The right coronary artery then directly enters the right atrioventricular groove, whereas the main stem of the left coronary artery runs a short course before dividing to become the anterior interventricular and circumflex arteries. These arteries can have an anomalous origin from either the aorta or pulmonary trunk; their branches can have various anomalous origins relative to arterial pedicles. Other abnormal situations include myocardial bridging, abnormal communications, solitary coronary arteries, and duplicated arteries. Understanding of these variations is key to determining those anomalous patterns associated with sudden cardiac death. In the most common variant of an anomalous origin from the pulmonary trunk, the main stem of the left coronary artery arises from the sinus of the pulmonary trunk adjacent to the anticipated left coronary arterial aortic sinus. The artery can, however, arise from a pulmonary artery, or the right coronary artery can have an anomalous pulmonary origin. The key feature in the anomalous aortic origin is the potential for squeezing of the artery, produced by either the so-called intramural origin from the aorta, or the passage of the abnormal artery between the aortic root and the subpulmonary infundibulum.     

Tight mitral stenosis with normal unilateral pulmonary capillary pressure

There are few reports of severe mitral stenosis with normal pulmonary artery wedge pressure. In order to illustrate this problem we present two adult patients with these abnormalities. Both patients had intact interatrial septum with partial anomalous pulmonary venous drainage, one to the inferior vena cava (The Scimitar Syndrome) and the other to the superior vena cava. In the first case the right pulmonary artery wedge pressure was 11 mm Hg. In the second case this pressure was only 8 mm Hg. Pulmonary arteriograms are of paramount importance in the diagnosis and surgical management. In both cases the surgical procedure with mitral commissurotomy and transplant of the anomalous pulmonary veins to the left atrium was successful.     

Pulmonary circulation in pulmonary atresia associated with the asplenia cardiac syndrome.

OBJECTIVE. The goal of this study was to determine the patterns of the pulmonary circulation in patients with pulmonary atresia and asplenia. BACKGROUND. The asplenic cardiac syndromes characteristically have complex cardiac anomalies including pulmonary stenosis or atresia. Definition of the pulmonary artery circulation and pulmonary venous connections is needed for consideration of surgical procedures. METHODS. In 35 patients, the sources of pulmonary blood flow, anatomic features of pulmonary arteries and pulmonary venous connections were determined from angiograms or autopsy specimens. RESULTS. The main pulmonary artery was absent or hypoplastic in 91% of patients; most had a ductus arteriosus. The right and left pulmonary arteries were confluent in 90% and usually of normal size (right 71%, left 63%). Total anomalous pulmonary venous connections were present in 38%. CONCLUSIONS. The anatomic features of the pulmonary arteries in pulmonary atresia associated with the asplenic cardiac syndrome are usually favorable for palliative surgical procedures. Total anomalous pulmonary venous connection may exist as a complicating factor.     

A case report of intralobar sequestration with a Mycobacterium tuberculosis infection limited to the sequestrated lung]

A 22-year-old woman was referred to our hospital suffering from repeated exacerbation of infiltrates confined to the medial portion of the right lower lung lobe, suggestive of pulmonary sequestration. Angiography revealed an anomalous vessel originating from the left side of the descending aorta, flowing behind it into the right lower lobe, the blood being returned to the pulmonary veins. A resection of the right lower lobe was conducted via thoracotomy. However, since there was no clear normal lung-pleura interface, this was classified as Pryce II type intralobar sequestration. Epithelioid granulomas with associated caseation necrosis confined to the sequestrated lung were confirmed by a polymerase chain reaction as Mycobacterium tuberculosis. Following oral antituberculosis drug administration, the patient recovered uneventfully. Superimposition of tuberculosis confined to a sequestrated lung portion is extremely rare. The presence of the aberrant artery led us to conclude that the intralobar sequestration must have been congenital.