Unilateral Absence Of Pulmonary Artery Research Articles

Repair of Unilateral Absence of Right Pulmonary Artery With Contralateral Pulmonary Artery Autograft Interposition in an Infant

Unilateral absence of the pulmonary artery (PA) is a rare congenital abnormality. Although a traditional 2-stage approach with a preceding systemic-to-PA shunt followed by a definitive repair is mandatory in patients with absent extrapericardial pulmonary arteries, more creative solutions are necessary when 1-stage repair is concerned. We present anovel technique for repairing the absent intrapericardial right pulmonary artery (RPA) with a contralateral PA autograft segment interposition. We believe that this technique using solely autologous tissue is effective in patients in whom the intrapericardial segment of the PAs is absent.

Isolated right pulmonary artery agenesis with aplasia of right upper lobe and with anomalous arterial supply from celiac axis, anomalous venous drainage

Pulmonary artery agenesis is the complete absence of the right or left pulmonary artery. This lesion is commonly associated with other cardiac anomalies and is usually diagnosed incidentally. Unilateral absence of pulmonary artery with lung hypoplasia is very rare. Ipsilateral hypoplastic or absent central pulmonary artery in these patients will have a systemic arterial supply to peripheral pulmonary arteries of the affected lung from the descending thoracic or upper abdominal aorta. These findings are usually characterized as pulmonary veno lobar syndrome or scimitar syndrome when associated with ipsilateral total or partial anomalous pulmonary venous return from the lower lobe which drains into the inferior vena cava or less commonly to the hepatic, azygous, or portal vein, or into the right atrium and lung agenesis/hypoplasia.

Unusual Etiologic Factor of Massive Hemoptysis: Unilateral Absence of Pulmonary Artery

Unusual Etiologic Factor of Massive Hemoptysis: Unilateral Absence of Pulmonary Artery

Management of Tetralogy of Fallot With Unilateral Absence of Pulmonary Artery

Tetralogy of Fallot with unilateral absence of pulmonary artery (PA) is a rare congenital anomaly that has been reported in isolated case reports and small case series. There is no well-defined treatment algorithm for these patients, and repair has been associated with high mortality, although survival is improving in the more recent era. Recent reports suggest strict case selection criteria based on PA dimensions and size of the left ventricle.

Imaging Features of Isolated Unilateral Pulmonary Artery Agenesis

To highlight the variation in imaging and clinical manifestations of four cases of isolated unilateral absence of pulmonary artery presented in varied ages. Four patients with unilateral pulmonary artery agenesis were referred to our institution They underwent a series of investigations, including chest radiography, computed tomography and magnetic resonance imaging. Two of the four patients had absence of the right main pulmonary artery, while the remaining two patients had absence of the left main pulmonary artery. All patients showed a restrictive defect on pulmonary function tests. Radiography, CT and MRI confirmed the anatomy and the presence of multiple collaterals. Isolated unilateral pulmonary artery agenesis has a non-specific presentation. Awareness of this condition can lead to earlier diagnosis, with cross-sectional imaging making an important contribution.

Double outlet right ventricle with unilateral absence of left pulmonary artery

Congenital absence of unilateral pulmonary artery, either as a primary defect or in combination with other congenital cardiovascular malformations, is very rare. Double outlet right ventricle pathology in combination with unilateral absence of pulmonary artery has only been reported once in the literature. In this report, we present our experience with double outlet right ventricle with unilateral absence of left pulmonary artery in a 3-year-old female patient who underwent a palliative procedure and was scheduled for correction.

Tetralogy of Fallot with unilateral absent pulmonary artery

Tetralogy of Fallot is a common congenital cardiac malformation. A rare subgroup includes unilateral absence of the pulmonary artery, either the left or the right main branch. The literature lacks an established treatment for these cases, and surgical options carry certain mortality and morbidity. There were five patients who had single pulmonary artery and received surgical treatment among the 126 patients with the diagnosis of Tetralogy of Fallot, who were admitted to our institution between July, 2010 and November, 2011. All the patients were male. Ages ranged between 12 months and 8 years. The mean body mass index was 17.1 plus or minus 3.4 kilograms per square metre. Pulmonary artery Nakata index, Nakata index Z-score, and the McGoon index were used for the quantitative assessment of the pulmonary artery and to determine the surgical strategy. Urgent modified Blalock-Taussig shunt operations were performed in two patients with very low oxygen saturation and haemodynamic instability. These patients are scheduled for corrective procedures on an elective basis. There was one patient who received an elective shunt procedure; however, the post-operative course was complicated with the overflow phenomenon and the patient underwent total correction with a check-valved patch used to close the ventricular septal defect. The patient required extracorporeal membrane oxygenator support in the post-operative period. There were two patients who underwent total correction of the pathology uneventfully. Mortality did not occur. Mean durations of hospital stay and follow-up were 14 plus or minus 13.4 days and 184.5 plus or minus 89.3 days, respectively. Our modest series with Tetralogy of Fallot with unilateral absent pulmonary artery indicates the feasibility of surgical correction in patients with appropriate unilateral pulmonary artery size and palliative procedures when the pulmonary artery size is smaller than that predicted for the age. Multi-centre long-term data of larger series are warranted in order to establish a treatment protocol.

Bullous lung disease and bronchiectasis in unilateral absent right pulmonary artery

Unilateral absence of pulmonary artery is an uncommon congenital disorder. We report a case of unilateral absence of the right pulmonary artery in an adult without any other cardiovascular anomalies. We describe the clinical and radiologic presentations of this disease. Computed tomography scan and Surgery enabled us to confirm the diagnosis precisely, which was successfully treated with a right pneumonectomy for right-sided bullous lung disease and bronchiectasis. He is doing well at 2 year follow-up.

Isolated unilateral absence of pulmonary artery mimicking chronic pulmonary embolism at chest X-ray: multidetector-row CT angiographic findings

Unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly which can seldom be isolated (1:200,000) and incidentally discovered in adulthood. We describe the case of a 54-year-old male patient who was found to have isolated UAPA (IUAPA) during the clinical and radiological investigation of a single episode of hemoptysis. Although abnormal, chest X-ray findings differed considerably from those previously reported and the diagnosis was only achieved by multidetector-row computed tomography angiography and later confirmed by bed-side echocardiography. Further clinical and instrumental investigation revealed systemic hypertension (158/95 mmHg) and bilateral mild hydronephrosis which both remitted after transurethral prostatic adenomyomectomy.

Unilateral absence of right proximal pulmonary artery in the setting of severe pulmonary hypertension: considerations for surgical repair and perioperative management

We report a successfully treated case of unilateral absence of a pulmonary artery, associated with an atrial septal defect and chronic lung disease with severe pulmonary hypertension. Because this severe pulmonary hypertension could jeopardize postoperative hemodynamic, the ingenuity of surgical strategy was required. The atrial septal defect was left open as a safety "pop-off" valve, and prosthetic graft was chosen as a reconstructive material to avoid excessive dilatation from exposure to unpredictable postoperative pulmonary hypertension.

Unilateral Absence of Pulmonary Artery: An Uncommon Cause of Pulmonary Hypertensio

Unilateral Absence of Pulmonary Artery: An Uncommon Cause of Pulmonary Hypertensio

Congenital isolated unilateral absence of pulmonary artery and variants of collateral blood supply of the ipsilateral lung

Congenital isolated unilateral absence of pulmonary artery and variants of collateral blood supply of the ipsilateral lung

Unilateral absence of pulmonary artery in children: bronchovascular anatomy, natural course and effect of treatment on lung growth

Unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly with few published studies focusing on anatomy and outcome. To assess the bronchovascular anatomy, lung volume and growth in treated and untreated patients with UAPA. Eighteen children with UAPA (isolated: n = 12; associated with congenital heart disease: n = 6) were retrospectively studied to assess the vascularization and lung segmentation and to appraise lung volume evolution in treated and untreated patients. Age at presentation: 1 day to 6 years; mean follow-up duration 13.6 years. Reperfusion of the affected pulmonary artery was attempted in 10 children (younger than 6 months: n = 7; older than 6 months: n = 3). Bronchovascular lung segmentation was complete in all cases. In children treated before 6 months of age, lung volume normalized in 3 and remained normal in 3, and hypoplasia progression was noted in 1. Hypoplasia persisted in children treated after 6 months of age. In untreated children, lung hypoplasia was unchanged in cases diagnosed after 7 months of age (n = 4) and progressive in cases diagnosed before 3 months (n = 4). In UAPA, lung anatomy and volume are normal at birth. Revascularization of the affected pulmonary artery before 6 months of age seems to allow optimal lung growth and prevent postnatal lung hypoplasia and development of collaterals.

Unilateral Absence of the Left Pulmonary Artery Accompanied by Right Lung Cancer

Unilateral absence of a pulmonary artery is a rare congenital anomaly that is often accompanied by other cardiovascular anomalies. Surgical treatment for this rare disease is usually performed only on the abnormal side. We report on a case of unilateral absence of a pulmonary artery that was complicated by contralateral primary lung cancer. We performed a right middle lobectomy despite the absence of the left pulmonary artery and detected changes in respiratory function during the operation. The present case provides information on the surgical tolerability and predictable morbidity following lung resection of the normal side in unilateral absence of a pulmonary artery.

Unilateral Absence of a Left Pulmonary Artery

Unilateral absence of a pulmonary artery is a rare anomaly, with only 108 case reports since the first observation by Frantzel in 1868.1 Absence of a left pulmonary artery occurs at an even more infrequent rate, accounting for one third of all unilateral absence of a pulmonary artery.2 A late presentation in life, as with the patient described below, is uncommon, and delayed diagnosis may be the result of atypical symptoms earlier in life that went unrecognized. Such an anomaly can present with a wide array of symptoms including chest pain, shortness of breath, hemoptysis, fatigue, and decreased functional capacity for physical exertion. Therapeutic options are many and include surgical resection of lung lobe/tissue and medical therapy with endothelin receptor antagonists, prostacyclin, and nitric oxide. Despite evidence surrounding the vast array of therapeutic options, there has been no published data describing combination therapy using bosentan and warfarin. A 58-year-old white woman presented for an outpatient evaluation of dyspnea on exertion. The patient initially described progressive increase …

Surgical Correction of Tetralogy of Fallot With Unilateral Absence of Pulmonary Artery

Tetralogy of Fallot with unilateral absence of the pulmonary artery is a rare congenital heart defect that still represents a surgical challenge. The purpose of this study is to summarize our experience of surgical treatment of this complex lesion. From 1983 to 2003, 27 patients with tetralogy of Fallot and unilateral absence of the left (n = 25) or right (n = 2) pulmonary artery underwent different surgical interventions. The age of patients ranged from 40 days to 37 years (median, 5.3 years). Pulmonary arterial Nakata index and Nakata index Z-score were used for the quantitative assessment of the contralateral pulmonary artery. Twenty patients underwent various palliative procedures, namely Blalock-Taussig or Gore-Tex shunt, transluminal balloon pulmonary valvuloplasty, and reconstruction of right ventricular outflow tract without ventricular septal defect closure. At a median interval of 3.6 years after palliation, 13 patients underwent complete repair of tetralogy of Fallot. In the other 7 patients, complete repair was performed as a primary intervention. Hospital mortality after palliation and after a complete repair was the same and reached 5%. Sixteen patients with the Nakata index greater than 200 mm2/m2 and Z-score greater than -4 survived after a complete repair. One of 4 patients with Nakata index less than 200 mm2/m2 and Z-score less than -4 died after surgery. Majority of patients with tetralogy of Fallot and unilateral absence of the pulmonary artery require palliative intervention as a first step of surgical treatment. Nakata index greater than 200 mm2/m2 and Nakata index Z-score greater than -4 are criteria for a successful complete repair.

Unilateral Absence of the Pulmonary Artery Incidentally Found After an Episode of Hemoptysis

A 29-year-old woman was admitted to our hospital because of hemoptysis and dyspnea. A chest radiographic study revealed an enlargement of the right lung hilum, and the left pulmonary artery could not be clearly visualized on contrast-enhanced CT scan. Both perfusion scintigraphy and pulmonary angiography confirmed the absence of the left pulmonary artery, and agenesis of the right upper pulmonary artery. Her clinical status has been stable for the previous two years of follow-up, even though she required long-term oxygen therapy. This is a rare case of a young woman with the unilateral absence of the left pulmonary artery incidentally found after an episode of hemoptysis.

Surgery for unilateral absence of pulmonary artery using autologous tissue

A 7-year-old girl with unilateral absence of the pulmonary artery underwent autologous tissue limited reconstructive surgery. The proximal portion of the artery was reconstructed by rotating a reverse U-shaped cut opposite the pulmonary arterial wall and covering the anterior surface with autologous pericardium. Follow-up catheterization at 5.8 years after surgery revealed no stenosis. This procedure could become one of the preferred methods for this unusual clinical condition.

Unilateral absence of pulmonary artery and ventricular septal defect in an infant

A 2-month-old boy was diagnosed as having a rare combination of absence of left pulmonary artery and ventricular septal defect. He underwent intracardiac repair at the age of 8 months. Reconstruction of the left pulmonary artery was impossible because it could not be visualized through a median sternotomy. Although his early postoperative course was uneventful, he needed ipsilateral pneumonectomy 3 months later because of unremitting hemoptysis. Hilar left pulmonary artery, which used to exist, was not found even by histologic examination.

Surgical Repair of Patients With Tetralogy of Fallot and Unilateral Absence of Pulmonary Artery

Background. Patients with tetralogy of Fallot and unilateral absence of pulmonary artery are a high-risk group for whom there is no consensus on the correct approach to medical management. The purpose of this report is to review a 29-year experience in the treatment of those patients. Methods. Between May 1966 and February 1995, 2,511 patients underwent correction of tetralogy of Fallot in our department, 24 of those patients with unilateral absence of pulmonary artery (20 had absence of the left pulmonary artery, 4 had absence of the right pulmonary artery). Valved conduits were used in 9 patients, right ventricular patches were used in 4 patients, and transannular patches with a monocusp that was made of the patient’s pericardium were used in 11 patients. Results. There were two operative deaths; both were in patients with hypoplasia of the left ventricle. All survivors had good early and late results. Conclusions. A right ventricular patch should be used in patients with tetralogy of Fallot and infundibular stenosis; a transannular patch with a monocusp should be used in patients with tetralogy of Fallot and stenosis of the left or right pulmonary artery’s origin as well as the pulmonary trunk. A homograft valved conduit is suitable for patients with anomalous coronary artery or pulmonary atresia.