Absence Of Pulmonary Artery Research Articles

Isolated Unilateral Absence of Pulmonary Artery: Adult Manifestations of a Congenital Disease

SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Vascular Disease I SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Unilateral absence of pulmonary artery (UAPA) is a rare congenital disorder. In the absence of other lesions, is survivable into adulthood. Adult patients can present with chest pain (CP), dyspnea on exertion (DOE), recurrent infections, or hemoptysis. Clinical diagnosis is difficult without radiographic support. CASE PRESENTATION: 56 year-old female former smoker with moderate obstructive lung disease presented for second opinion after multiple hospital admissions for CP, DOE and recurrent bronchitis. Hyper-inflated left lung, right mediastinal shift, and absent right hilar shadow noted on chest x-ray (CXR). CT angiogram revealed hypoplastic right lung with right greater left centrilobular emphysema, right nodularity, ground glass, and abrupt cessation right pulmonary artery. Right and left heart catheterization revealed mean pulmonary pressure of 27, and large arterial fistula between the left circumflex coronary artery and several branches of the right pulmonary vasculature. CT coronaries showed proximal interruption of the right pulmonary artery with collateral vasculature recruited from SA nodal branch of the left circumflex, bronchial, internal mammary, intercostal, and phrenic arteries. DISCUSSION: Adult patients with isolated UAPA are often asymptomatic. Common presentations are pulmonary infections, CP, DOE, hemoptysis or pulmonary hypertension (PAH). CXR shows ipsilateral contracted lung with mediastinal, tracheal shift toward the affected side, absent hilar shadow, and contralateral lung hyperinflation. Diagnosis is confirmed with contrast-enhanced CT. Recurrent infections can be secondary to poor blood flow to the affected lung, reduced delivery of inflammatory cells and impaired ciliary function. Decreased blood flow can also result in alveolar hypocapnia causing secondary bronchoconstriction and mucus trapping. PAH results from increased blood flow to the contralateral pulmonary artery, increased shear endothelial stress, and release of vasoconstrictors. Our patient's chest pain was felt not due to coronary steal as cardiac stress testing was negative. There is no clear consensus on treatment for UAPA other than symptom management. CONCLUSIONS: Although isolated UAPA is a congenital abnormality, it can remain undetected until adulthood. Clinicians need to be aware of characteristic CXR findings that may suggest UAPA. . Reference #1: Harke, A et al. Isolated Unilateral Absence of a Pulmonary Artery: A Case Report and Review of the Literature.” CHEST 2002; (122.4) 1471-477. Reference #2: Yiu MWC et al. Radiological features of isolated unilateral absence of the pulmonary artery. J HK Coll Radiol. 2001;4:277-280. Reference #3: Kruzliak, P et al. Unilateral Absence of Pulmonary Artery: Pathophysiology, Symptoms, Diagnosis and Current Treatment. Archives of Cardiovascular Diseases 2013; 106.8-9: 448-54 DISCLOSURE: The following authors have nothing to disclose: Santosh Reddy, Kimberly Cao No Product/Research Disclosure Information

Tracheal Bronchus Associated With Unilateral Absence of Pulmonary Artery and Recurrent Pneumonia

Introduction: Tracheal bronchus or pig bronchus is a rare congenital lung anomaly .In this condition, an ectopic bronchus arises from the lateral wall of the trachea, more commonly from right side. Although pig bronchus is usually asymptomatic, however in some patients it is presented with recurrent pneumonia and segmental atelectasis. Case Presentation: The patient was a five-month-old female referred to the clinic because of pneumonia with inappropriate response to antibacterial treatment and previous history of recurrent pneumonia. On chest X-ray, she had reduced volume right lung with mediastinal shift to the right side. Thorax computed tomography scan (CT scan) revealed pneumonitis. On bronchoscopy, there was no foreign body, but purulent exudates and fibrin were observed in her right lung and possibility of tracheal bronchus was not proposed. Although axial views of CT scan did not determine any anatomic anomaly, the coronal view reconstruction of her first CT scan revealed tracheal bronchus above the carina on the right side. The images of axial cuts of CT scan re-inspect one more time; it was interesting that it had some evidence of tracheal bronchus. Conclusions: Recurrent pneumonia in right upper lobe sensitized physicians about the possibility of congenital anomalies particularly tracheal bronchus and coronal view reconstruction of CT scan should be requested.

Novel Approach to Construct Absent Intrapericardial Branch Pulmonary Arteries

Unilateral or bilateral absence of branch pulmonaryarteries is a rare congenital heart defect. Variousreconstructive techniques have been previously described, each with its inherent limitations. Wepresent a novel technique of constructing a branch pulmonary artery that maintains potential for growth.

Surgical Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals With Absent Intrapericardial Pulmonary Arteries

One anatomic variant of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is characterized by the absence of intrapericardial pulmonary arteries. This anatomy obviates the possibility of incorporating the pulmonary arteries for reconstruction or palliative procedures. The purpose of this study was to evaluate the surgical results in patients undergoing repair of PA/VSD/MAPCAs with absent pulmonary arteries. This was a retrospective review of 35 patients who underwent surgical repair of PA/VSD/MAPCAs with absent pulmonary arteries between 2007 and 2014. The median age at the time of surgery was 3.4 months, and the median weight was 4.9 kg. All patients underwent unifocalization of MAPCAs, with an average of 3.5± 1.4 MAPCAs per patient. Twenty-eight of the 35 patients (80%) underwent complete single-stage surgical repair, including unifocalization of MAPCAs, VSD closure, and right ventricle to pulmonary artery conduit. After complete repair, the average right ventricular to aortic pressure ratio was 0.33 ± 0.07. There were no deaths in this subgroup. Seven patients (20%) were not deemed suitable candidates for VSD closure after their unifocalization procedure, and therefore underwent palliation with a central shunt. There was 1 operative death and 1 interim death. Three patients have subsequently undergone complete repair, and 2 are awaiting further evaluation and treatment. The majority of patients with PA/VSD/MAPCAs and absent pulmonary arteries can undergo complete single-stage repair with satisfactory postoperative hemodynamics. These results suggest that unifocalization of MAPCAs can provide a reasonable pulmonary vascular bed in the absence of intrapericardial pulmonary arteries.

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.

Hypoplasia of left pulmonary artery: A rare congenital heart disease

Unilateral hypoplasia or absence of pulmonary artery is a rare pulmonary vascular anomaly that is usually associated with other cardiac anomalies like Tetralogy of Fallot or cardiac septal defects, but can also occur as an isolated anomaly. We present a case of 6-monthold infant who presented with recurrent respiratory tract infection. On work up, chest X-ray showed ipsilateral small hemithorax with hyperluscency in the left lung field. Findings were confirmed with 2D echocardiography and pulmonary angiography which were suggestive of hypoplasia of left pulmonary artery.

Pulmonary arterial hypertension in a patient with unilateral pulmonary artery absence (RCD code: II-2A.1)

Pulmonary artery absence is a rare congenital defect, occurring in 1 out of 100 000 individuals. In its isolated unilateral form only one in three cases concerns the left artery. The first symptoms usually appear in young age. Pulmonary hypertension associates this defect in 44% of patients. We present a 41-year-old female who was referred to our institution in January 2014 for further examination with preliminary diagnosis of central pulmonary embolism based on CT angiograms. There were also echocardiographic signs of pulmonary hypertension. The lady was severely dyspnoeic and her complaints included palpitations and dry cough. She had been previously treated for chronic bronchitis and sinusitis, allergic rash and anemia. In 2013 she also suffered left-sided pneumonia. Until December 2013 she had been a smoker. She gave birth thrice, all the pregnancies went uncomplicated. We established a final diagnosis of isolated unilateral left pulmonary artery absence without lung hypoplasia and precapillary pulmonary hypertension based on RHC. Therapy with sildenafil 20 mg tid was initiated and it was escalated shortly after to sildenafil plus iloprost due to the lack of clinical and hemodynamic improvement. Finally, being on dual therapy for 14 months now, the patient responded to treatment with symptomatic improvement from WHO class IV to class II. Furthermore there is a moderate regression of pulmonary vascular resistance and some improvement in cardiac index as documented in a follow-up RHC. The patient is listed for bilateral lung transplantation.

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.

Pulmonary underdevelopment malformation with cleft lip and palate – A case report

Pulmonary underdevelopment malformations are rare. Ipsilateral facial defects and lung aplasia especially on right side are reported in literature. We present an adolescent with right cleft lip and palate incidentally detected to have aplasia of left lung with absent pulmonary artery after two general anaesthesia procedures. Computed tomogram is a good non invasive technique for a definitive diagnosis.

Variations of pulmonary arteries and other associated defects in Tetralogy of Fallot.

BackgroundThe objective of study was to determine pulmonary artery variations and other associated cardiac defects in patients with Tetralogy of Fallot. This cross-sectional, descriptive study was carried out at The Children's Hospital and the Institute of Child Health, Lahore, from January 2006 to December 2012. All patients with Tetralogy of Fallot, who underwent cardiac catheterization during this period, were included. Standard cine-angiograms were done to record the pulmonary artery sizes and associated cardiac defects.DescriptionA total of 576 patients with Tetralogy of Fallot were catheterized. Pulmonary Artery abnormalities were present in 109 (18.92%) patients. The commonest abnormality was isolated Left Pulmonary Artery stenosis (n = 60, 10.4%) followed by supra-valvular stenosis (n = 9, 1.6%). Left Pulmonary Artery was absent in seven patients(1.2%), while 1 patient (0.2%) had both absent right and left Pulmonary Arteries with segmental branch pulmonary arteries originating directly from Main Pulmonary Artery. Associated cardiac lesions included right aortic arch in 72 (12.5%), additional muscular Ventricular Septal Defect in 31 (5.4%), Patent Ductus Arteriosus in 31 (5.4%), bilateral Superior Vena Cava 36(6.2%), Atrial Septal Defect 4(0.7%) and Major Aortopulmonary Collateral Arteries in 75(13%) patients. Significant coronary artery abnormalities were present in 28(4.9%) children.ConclusionPulmonary artery abnormalities were present in 18.92% of patients with Tetralogy of Fallot. Isolated Left Pulmonary Artery origin stenosis was the most common abnormality. Significant associated cardiac lesions including Patent Ductus Arteriosus , additional muscular Ventricular Septal Defect, coronary artery abnormalities, bilateral Superior Vena Cava, Atrial Septal Defect and Major Aortopulmonary Collateral Arteries were present in one-third of the patients.

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.

Letter to the Editor Rare cardiovascular anomaly: congenital unilateral absence of the pulmonary artery

Letter to the Editor Rare cardiovascular anomaly: congenital unilateral absence of the pulmonary artery

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.

Detection of pulmonary arterial morphology in tetralogy of Fallot with pulmonary atresia by computed tomography: 12 years of experience

Our aim was to evaluate the feasibility of using computed tomography (CT) to define the pulmonary artery anatomy in patients with tetralogy of Fallot and pulmonary atresia (TOF-PA). We retrospectively reviewed 110 patients with TOF-PA between 1995 and 2008. Those who received cardiac catheterization and surgery within 3 months of their CT examinations were enrolled. Based on Dr. Somerville's classification, the pulmonary arterial pattern was determined, including identifiable pulmonary trunk (type I), the presence of both left and right pulmonary arteries without trunk (II), only left or right pulmonary artery present (III), and absent intrapericardial pulmonary arteries (IV). The accuracy of both imaging modalities was evaluated with operation findings as the golden standard. The effective radiation doses and adverse events were also recorded. In the 64 eligible patients (median age, 23 months), CT and catheterization demonstrated accurate pulmonary arterial morphology in 60 (60/64) and 53 (53/64) TOF-PA patients, respectively. Thirty-two of 35 type I patients were correctly identified by CT, whereas 26 were correctly identified by catheterization (p = 0.03). Of the 20 type II TOF-PA patients, 19 were diagnosed by CT, whereas 18 were diagnosed by catheterization. CT and catheterization both successfully defined six type III and three type IV patients. The median calculated radiation doses caused by CT and catheterization were 4.5 and 5.6 mSv, respectively (p > 0.05). For patients with TOF-PA, CT could accurately delineate pulmonary arterial morphology with the same level of accuracy as cardiac catheterization. Therefore, CT can be considered a reasonable diagnostic alternative for such patients.