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Fulltext Pneumonectomy for congenital isolated unilateral pulmonary artery agenesis

Britton J, Sachithanandan A, Srinivasan L, Ghosh S

Med J Malaysia, 2011 Oct;66(4):363-4.

Unilateral pulmonary artery agenesis (UPAA) is a rare congenital anomaly usually diagnosed in infancy due to associated cardiovascular malformations. We report a rare case of isolated right UPAA that presented atypically in adulthood with massive haemoptysis requiring a pneumonectomy. This case highlights the importance of maintaining a high clinical suspicion, the role of CT angiography and a multi disciplinary approach. Optimal management is often surgical however bronchial artery embolization (BAE) remains a useful adjunct.

Matched MeSH terms: Pulmonary Artery/abnormalities*

Fulltext Severe and fatal postoperative bronchospasm in a child with a pulmonary artery sling

Norzila MZ, Azizi BH, Mazeni A, Ahmad S, Devadass P, Lokman S

Med J Malaysia, 1994 Jun;49(2):182-4.

PMID: 8090102

We report a case of left pulmonary artery sling in a child who also had duodenal atresia. He was admitted for respiratory failure requiring prolonged respiratory support due to a bronchiolitis-like illness at two months of age. Diagnostic procedures confirmed the presence of left pulmonary artery sling. He had a corrective procedure which relieved the compression. However postoperatively he had intermittent episodes of severe bronchospasm in addition to a persistent airway obstruction. Finally one such episode of severe bronchospasm did not respond to medical and resuscitative therapy and the baby succumbed. To our knowledge no case of left pulmonary artery sling has been described previously in a Malaysian child. This case also highlights the postoperative airway problems that may be encountered.

Matched MeSH terms: Pulmonary Artery/abnormalities*

Fulltext Development of pseudo-subarachnoid hemorrhage secondary to hypoxic-ischemic injury due to bleeding pulmonary arterio-venous malformation

Md Noh MSF, Abdul Rashid AM

BMC Neurol, 2018 Sep 28;18(1):157.

PMID: 30266082 DOI: 10.1186/s12883-018-1161-x

BACKGROUND: The computed tomography (CT) finding of a pseudo-subarachnoid hemorrhage (SAH) may lead the treating physician into a diagnostic dilemma. We present a case of a pseudo-SAH in a patient with post-resuscitative encephalopathy, secondary to a newly diagnosed bleeding pulmonary arterio-venous malformation (AVM).

CASE PRESENTATION: A 19-year-old female presented acutely with massive hemoptysis. Cardiopulmonary resuscitation (CPR) followed, and the patient was subsequently intubated for airway protection with intensive care unit (ICU) admission. Urgent CT angiography of the thorax showed a bleeding pulmonary AVM, with evidence of hemothorax. Non-contrasted cranial CT initially revealed cerebral edema. Day 3 post admission, repeat cranial CT showed worsening cerebral edema, with evidence of pseudo-SAH. Patient passed away the next day.

CONCLUSIONS: Pseudo-SAH, if present, carries a poor prognosis. It should be recognized as a potential CT finding in patients with severe cerebral edema, due to various causes. The diagnosis is vital, to avoid wrongful treatment institution, as well as determination of cause of death.

Matched MeSH terms: Pulmonary Artery/abnormalities*

Congenital cyanotic heart disease with situs inversus totalis: an autopsy case report

Dayapala A, Kumar V

Am J Forensic Med Pathol, 2009 Jun;30(2):171-4.

PMID: 19465810 DOI: 10.1097/PAF.0b013e3181875a79

Sudden deaths because of congenital heart diseases are infrequently referred to the forensic pathologist for autopsy. Many of such deaths, if already diagnosed are released directly from the hospital without autopsy. Even forensic pathologists face a few difficulties in performing the autopsy on such infrequent cases, as they are not always updated with the anatomy of anomalies. While dealing with such cases, the concerned forensic pathologist is compelled to refer literature and textbooks again to understand the nature of developmental defects. This is especially so when dealing with cases of situs inversus accompanied by transposition of great arteries and other congenital cardiac abnormalities and variants such as single ventricle, double outlet right ventricle, Taussig-Bing variety etc. In the present case also, situs inversus with transposition of great vessels and other anomalies have been noted and studied.

Matched MeSH terms: Pulmonary Artery/abnormalities

Large anomalous systemic arterial supply to the left lung without pulmonary sequestration: a rare cause of heart failure in a child

Wong MN, Joshi P, Sim KH

Pediatr Cardiol, 2009 Jan;30(1):85-6.

PMID: 18663510 DOI: 10.1007/s00246-008-9287-z

A 10-month-old boy was referred for tachypnea and heart murmur. An echocardiogram showed unexplained left heart dilation without evidence of an intracardiac shunt. A 64-slice computed tomographic contrast-enhanced angiography showed a large tortuous anomalous artery arising from the descending thoracic aorta and supplying the lower lobe of the left lung. The venous return into the left atrium was normal. The affected lobe had normal lung parenchyma, and its bronchial tree was connected normally with the left main bronchus. Hence, it was not a sequestrated lobe. The boy underwent surgical lobectomy of the left lower lobe and improved. Anomalous arterial supply of a lobe without sequestration of its bronchial tree is a rare pathologic entity. It also is a very rare cause of congestive heart failure in children. Computed tomographic angiography was a useful tool for evaluation of the intrathoracic anomalous vessel in this case.

Matched MeSH terms: Pulmonary Artery/abnormalities*

Fulltext Pulmonary arteriovenous malformation: a rare cause of cyanosis in a child

Sharifah AI, Jasvinder K, Rus AA

Singapore Med J, 2009 Apr;50(4):e127-9.

PMID: 19421665

Pulmonary arteriovenous malformations are rare vascular anomalies. We report a 12-year-old girl who presented with exertional dyspnoea, cyanosis and clubbing since the age of five years, and multiple pulmonary arteriovenous malformations. Computed tomography pulmonary angiogram showed a large pulmonary arteriovenous malformation at the lower lobe of the right lung. Pulmonary angiogram showed a large right lung arteriovenous malformation and two small left lung arteriovenous malformations. The multiple arteriovenous malformations were occluded with Gianturco coils. She is now asymptomatic and on regular follow-up.

Matched MeSH terms: Pulmonary Artery/abnormalities*

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