Pulmonary pseudosequestration receiving arterial supply from the right coronary artery: a case report

A 66-year-old female had been treated by hemodialysis since 1996. She was admitted to our hospital with acute pneumonia in January 2001. During admission, ischemic heart disease was identified. Her condition deteriorated and organic pneumonia of the right middle lobe progressed. She recovered after 6 months and coronary arteriography was performed. A 90% stenosis was detected at the ostium of the right coronary artery. An aberrant tortuous artery arose from the distal sinus node artery, and drained into the lung network, but also partially drained to the right segmental pulmonary artery branch. The diagnosis was significant stenosis of the right coronary artery, and pulmonary pseudosequestration or pulmonary sequestration receiving arterial supply from the sinus node artery. Surgical revascularization, ligation of the aberrant artery, and partial resection of the right middle lobe were performed. However, intraoperative findings did not identify the pulmonary sequestration. This rare case of pulmonary pseudosequestration received the arterial supply from the sinus node artery, originating from the right coronary artery with a significant stenotic lesion, and developed without recurrent pneumonia.     

Intralobar sequestration with tuberculous infection confined to the sequestrated lung

A 22-year-old female was referred to the hospital suffering from a persistent, non-productive cough and repeated exacerbations of a right lower zone infiltrate suggestive of pulmonary sequestration. Angiography revealed an aberrant artery that originated from the left side of the descending aorta, crossed the aorta anteriorly, flowed through the right lower lobe and drained into the right inferior pulmonary vein. Right lower lobectomy was conducted by open thoracotomy. Gross examination revealed mucoid impaction of bronchi in the sequestration. Microscopy demonstrated a clear boundary between sequestrated and normal lung, as well as caseating epitheloid granulomas confined to the sequestration. As the aberrant artery fed both sequestrated and adjacent normal lung and the boundary did not involve the pleura, the lesion was classified as a Pryce's type II intralobar sequestration. No acid-fast bacilli were observed, but homogenates of the sequestration were positive for Mycobacterium tuberculosis by polymerase chain reaction. The patient was treated with isoniazid and rifampicin daily for 9 months, and 6 years later her clinical status remained excellent. Tuberculosis confined to a sequestration is extremely rare and characteristics of the aberrant artery suggested this intralobar sequestration was likely to be congenital in origin.     

Partial anomalous origin of the left pulmonary artery

The pulmonary sling or aberrant left pulmonary artery has an incidence of 3% to 6% of all anomalies of the aortic arch system. We report a boy with coexistence of a normal and an anomalous left pulmonary artery. Associated congenital anomalies were coarctation, mitral stenosis, and imperforate anus. Cardiac catheterization and angiocardiography at the age of 5 months demonstrated an inconspicuous pulmonary trunk dividing into left and right branches. There was a normal right pulmonary artery, and a main left pulmonary artery supplying the left upper lobe, lingula, and anterior segments of the left lower lobe. In addition, an anomalous left lower lobe artery originated from the proximal right pulmonary artery, passed inferior to the tracheal bifurcation to the left supplying posterior segments of the left lower lobe. Chest X-rays and bronchoscopy could not detect any malformation of the trachea and pulmonary lobulation. There are only three reports of partial anomalous origin of the left pulmonary artery, and only one had a similar course of the accessory artery. This report is the first to present selective angiography and echocardiographic findings of the partial anomalous left pulmonary artery. The development of the partial anomalous pulmonary artery can be explained by the plexiform nature of the primary pulmonary vascular bed and can help to understand the embryology of the pulmonary arteries.     

Subclavian Steal Phenomenon Associated With Vascular Ring in an Infant Who Had a Prenatally Diagnosed Right Aortic Arch With an Atretic Aberrant Left Subclavian Artery

Vascular ring is a rare congenital anomaly in which the abnormal origin of the aorta or its branches and pulmonary arteries leads to encircling and compression of the trachea and esophagus. A right aortic arch (RAA) with an aberrant left subclavian artery is one of the most common forms of vascular ring. Here, we report a case of a prenatally diagnosed vascular ring resulting from an RAA with an aberrant left subclavian artery. When the infant was 7 months of age, the development of noisy breathing prompted further evaluation with cardiac magnetic resonance imaging that showed an atretic left subclavian artery associated with collateral retrograde flow from the left vertebral artery to the distal portion of the subclavian artery. Our findings indicate that an untreated RAA with an aberrant left subclavian artery may be associated with an increased risk of developing subclavian artery steal syndrome.     

Systemic arterial supply of the right lung with venous drainage to the pulmonary arterial circuit

In a girl suffering from "Scimitar syndrome", a rerouting of the scimitar vein was performed at the age of 6 years, but no embolisation of the aberrant systemic vessel was done. She presented with recurring respiratory problems 13 years later. An angiography revealed an invert flow from the aberrant systemic vessel via the right pulmonary artery into the left pulmonary artery. After pneumonectomy, she recovered well.     

Isolation of the left common carotid or left innominate artery

Isolation of the left common carotid or left innominate artery from the aortic arch is rare. A six week malformed infant with a right aortic arch had isolation of a left innominate artery and connection to the pulmonary artery by a left ductus arteriosus. A right ductus arteriosus had been ligated. Another infant with a right aortic arch and ostium primum atrial septal defect was shown to have an aberrant left subclavian artery arising from the lower descending aorta. The left common carotid artery filled retrogradely and drained into the pulmonary artery by a left ductus arteriosus. This abnormality has not been reported before.     

Isolation of the left common carotid or left innominate artery.

Isolation of the left common carotid or left innominate artery from the aortic arch is rare. A six week malformed infant with a right aortic arch had isolation of a left innominate artery and connection to the pulmonary artery by a left ductus arteriosus. A right ductus arteriosus had been ligated. Another infant with a right aortic arch and ostium primum atrial septal defect was shown to have an aberrant left subclavian artery arising from the lower descending aorta. The left common carotid artery filled retrogradely and drained into the pulmonary artery by a left ductus arteriosus. This abnormality has not been reported before.     

Surgical correction of isolated unilateral absence of right pulmonary artery

A 2-year-old girl with isolated unilateral absence of right pulmonary artery is described. Catheterization at 5 months demonstrated hypoplastic right pulmonary artery by pulmonary venous wedge angiography, and the patient underwent right Blalock-Taussig shunt and angioplasty of right pulmonary artery with autologous pericardial roll as an initial step. At 2 years, she underwent anastomosis of right pulmonary artery to main pulmonary artery with an autologous pericardial tube. Postoperative computed tomography showed a patent reconstructed right pulmonary artery.     

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.     

Right lung agenesis with left pulmonary artery sling

We report on a 2-month-old infant girl who had right pulmonary agenesis and an unusual course of the left pulmonary artery. Computed tomography and cardiac catheterization showed that the left pulmonary artery arose from the main pulmonary artery, crossing the midline, and reaching the left lung via an aberrant course between the esophagus and trachea. The coexistence of right pulmonary agenesis and left pulmonary sling is extremely rare. Unlike in other reports, our patient remained symptom-free and in good health, with normal growth and development until age 2 years, when she died from complications during an attack of bronchiolitis caused by respiratory syncytial virus.     

Unusual vascular ring in infant with pulmonary atresia and ventricular septal defect.

A 3-day-old infant investigated for cyanotic heart disease was found to have partial DiGeorge syndrome and pulmonary atresia with ventricular septal defect. The only source of pulmonary blood flow was a right-sided persistent ductus arteriosus which originated from an aberrant right subclavian artery. Such a vascular ring has not been described previously. Surgical treatment included a left-sided prosthetic shunt in infancy followed by resection of the ligamentum arteriosum and right subclavian artery during the second year of life.     

Hybrid Repair of an Aberrant Right Subclavian Artery with Kommerell's Diverticulum

This publication describes a hybrid endovascular and open surgical approach to treating a large aneurysm of an aberrant right subclavian artery (Kommerell's diverticulum). A 76-year old man presented with dysphagia lusoria due to a 3.5 × 3.0 cm aneurysm involving an aberrant right subclavian artery. The patient was treated by a thoracic aortic endograft, left subclavian artery de-branching (by its transposition to the left common carotid artery) and right subclavian artery revascularisation. This approach avoids the requirement for a thoracotomy or sternotomy needed with open surgical repair. At a 6 months follow-up assessment the aneurysm was shown to be thrombosed with no evidence of endoleak.     

Left common carotid artery arising from the pulmonary artery in a patient with DiGeorge syndrome.

A female infant, born at 33 weeks' gestation with tetralogy of Fallot, died of severe perinatal asphyxia 6 hours after birth. Necropsy disclosed two associated vascular anomalies: a right aortic arch with a left common carotid artery arising from the pulmonary artery (isolated left common carotid artery) and an aberrant left subclavian artery arising from the descending aorta. Agenesis of the thymus and parathyroid gland was also found, suggesting that the child also had DiGeorge syndrome. Origin of the left common carotid artery from the pulmonary artery is exceedingly rare. When planning surgical treatment it is important to be aware of the possibility of this anomaly occurring in association with congenital heart disease, particularly in the presence of tetralogy of Fallot, right sided aortic arch, or DiGeorge syndrome.     

Aberrant systemic artery-pulmonary venous fistula: diagnosis with Doppler imaging.

A 6-year-old asymptomatic girl presented with a continuous murmur at two different locations. Using Doppler imaging modalities, a small patent arterial duct and an aberrant systemic artery arising near the coeliac axis, piercing the right hemidiaphragm, and connecting to the right lower pulmonary vein were identified. Angiography confirmed the diagnosis and revealed additional pulmonary abnormalities. Doppler examination helped in planning appropriate angiographic projections and sites of the contrast medium injection.     

A case of preoperatively diagnosed primary pulmonary leiomyosarcoma]

A 29-year-old woman had been suffering from right back pain for 3 months. Chronic pulmonary thromboembolism was suspected and she was referred to our hospital. She presented with no risk factors for thromboembolism, and during the previous 6 months had lost 4 kg in body weight. Chest radiography showed nodular shadows in the lower field of the right lung. Contrast-enhanced computed tomography demonstrated a filling defect in the right pulmonary artery and nodular lesions in the lower field of the right lung, which were considered to be signs of pulmonary infarction. Absence of perfusion into the right lung was demonstrated by a perfusion scan. Right heart catheterization showed normal pressure in the pulmonary arteries, and pulmonary angiography showed an abrupt cutoff of the right pulmonary artery, which was similar to the finding of pulmonary thromboembolism. A transvenous catheter suction biopsy was performed in the right pulmonary artery and the histopathologic findings yielded a diagnosis of leiomyosarcoma. The patient underwent surgical resection under total cardiopulmonary bypass. A large tumor completely filled the right main pulmonary artery and invaded the posterior wall of the pulmonary trunk close to the left main pulmonary artery. Primary pulmonary leiomyosarcoma is a rare tumor and its prognosis is very poor. Radical surgical resection is the only effective treatment, but early diagnosis is very difficult. Transvenous catheter suction biopsy is a useful procedure for the early diagnosis of pulmonary artery sarcoma.     

Hemodynamically significant anomalies of the coronary arteries. Surgical aspects

In 78 (0.9%) of 8,283 patients without associated congenital heart disease studied by angiography, one or more major elements of the coronary arterial system originated in an ectopic manner. Symptoms attributable to the aberrant vessel more present in 20 of the 78 patients and in 15 patients these anomalies were treated surgically. The hemodynamically significant anomalies which lead to abnormalities of myocardial perfusion are of particular surgical importance. There are 4 major types: 1) aberrant origin of the left anterior descending branch from the pulmonary artery; 2) origin of the left coronary artery from the right aortic sinus; 3) origin of the left coronary artery from the pulmonary artery and 4) severe atherosclerotic occlusive disease in an aberrant vessel. The incidence, angiographic features and surgical aspects of these anomalies are discussed.     

Prenatal diagnosis of left pulmonary artery sling and review of literature

Left pulmonary artery sling (LPAS) is a very rare cause of large airway compression. In LPAS, the left pulmonary artery (LPA) arises from the proximal right pulmonary artery, coursing over the right mainstem bronchus, posterior to the trachea and anterior to the esophagus prior to reaching the left hilum. The aberrant course of the LPA results in anatomical obstruction of the right mainstem bronchus, the trachea, or both. Only a few reports present the prenatal features of LPAS. In this report, we present the prenatal diagnosis of a case of LPAS in one of a set of identical twins in which the only feature was that of an abnormal course of the LPA on 3‐vessel tracheal view. The cross‐sectional view at the level of three vessels which includes both pulmonary artery branches is useful to detect this abnormality. Color and power Doppler may be helpful as well.     

A reversible pulmonary artery band: preliminary experience

Pulmonary artery banding has become an infrequently used surgical technique. However, if a band was developed that could be relieved without the need for open heart surgery, it is likely that pulmonary artery banding would be used more frequently in the management of infants with congenital heart disease. Such a pulmonary artery band was placed in seven 1 week old mongrel puppies by using a loop of an absorbable suture material (Vicryl). One dog died at 2 months as a result of right ventricular failure. The remaining six dogs underwent cardiac catheterization and pulmonary balloon angioplasty at 6 months of age. After measuring pulmonary artery, right ventricular and aortic pressures and performing a right ventricular angiogram, balloon angioplasty of the band site was performed. A 20 mm balloon angioplasty catheter (Medi-Tech) was used in all dogs. Balloon angioplasty decreased right ventricular pressure from 101 +/- 19 to 42 +/- 3 mm Hg (p less than 0.05) and right ventricular systolic outflow tract gradient from 59 +/- 14 to 7 +/- 2 mm Hg (p less than 0.03), and increased the size of the band site from 8.7 +/- 0.03 to 14.9 +/- 0.5 mm (p less than 0.01). All dogs were recatheterized 2 months after angioplasty and were then killed for pathologic evaluation. At follow-up catheterization, right ventricular pressure, right ventricular outflow tract gradient and pulmonary artery size at the band site remained at the values obtained immediately after angioplasty. Postmortem examination demonstrated that there was no evidence of pulmonary artery damage. Although these studies are preliminary, they suggest that a reversible pulmonary artery band can be performed.     

Anomalous origin of coronary arteries: When one sinus fits all

A right coronary artery origin from the left coronary sinus and a left coronary origin from the right sinus although rarely encountered during routine cardiac catheterization, they represent two relatively common autopsy findings in young patients suffering sudden cardiac death. The interarterial course of the aberrant artery, between the aortic root and the pulmonary artery has been considered as a malignant variant, because of the higher risk of myocardial ischemia and sudden death. We present two rare cases of ectopic coronary origin from the opposite sinus of Valsalva.     

Anomalous origin of coronary arteries: When one sinus fits all

A right coronary artery origin from the left coronary sinus and a left coronary origin from the right sinus although rarely encountered during routine cardiac catheterization, they represent two relatively common autopsy findings in young patients suffering sudden cardiac death. The interarterial course of the aberrant artery, between the aortic root and the pulmonary artery has been considered as a malignant variant, because of the higher risk of myocardial ischemia and sudden death. We present two rare cases of ectopic coronary origin from the opposite sinus of Valsalva.