MyMedR

Beating the odds: a rare case of supracardiac total anomalous pulmonary venous return (TAPVR) in an adult patient

Talib N, Sayuti KA, Abdullah MS, Mohd Zain MR

BMJ Case Rep, 2018 Mar 05;2018.

PMID: 29507009 DOI: 10.1136/bcr-2017-221074

Total anomalous pulmonary venous return (TAPVR) is a rare congenital heart defect, and patients are usually symptomatic at a very young age. Survival to adulthood without surgical correction is extremely rare. We report a 33-year-old woman with a heart murmur and a history of a successful pregnancy. Echocardiogram revealed a large atrial septal defect with suspicious pulmonary vein anomaly. Chest radiograph demonstrated classical 'snowman' configuration. Cardiac catheterisation was consistent with anomalous pulmonary venous drainage. Cardiac CT confirmed supracardiac TAPVR, whereby all the pulmonary veins drain into the anomalous vein and finally to the superior vena cava. She remained asymptomatic and underwent a successful surgical repair.

Matched MeSH terms: Pulmonary Veins/abnormalities*; Pulmonary Veins/surgery

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 Veins/abnormalities*; Pulmonary Veins/radiography

An Unusual Type of Totally Anomalous Pulmonary Venous Connection to the Superior Cavoatrial Junction

Shamsuddin AM, Wong AR, Anderson RH, Corno AF

World J Pediatr Congenit Heart Surg, 2016 Jul;7(4):490-3.

PMID: 26795906 DOI: 10.1177/2150135115603330

A neonate with cyanosis at birth was found to have a rare type of totally anomalous pulmonary venous connection. The pulmonary veins entered a confluence posterior to the left atrial wall, which drained into the right superior cavoatrial junction. There were no other major structural defects and no evidence of isomerism. Because of the severe cyanosis, and the restrictive nature of the interatrial communication, we performed balloon atrioseptostomy to improve oxygenation. We then achieved successful surgical repair when the baby was aged 7 months.

Matched MeSH terms: Pulmonary Veins/abnormalities*

Value of CT pulmonary arteriography and venography in the evaluation of venous thromboembolism in a multiracial Asian population

Lee YJ, Zakaria R, Manaf ZA, Mohamed Z, Lee YY

Chin Med J (Engl), 2013;126(11):2049-53.

PMID: 23769556

There is increasing evidence that CT pulmonary arteriography and venography allow a better diagnostic yield for deep vein thrombosis (DVT) in suspected acute pulmonary embolism (PE). The aim was to investigate the value for using such an approach in a multiracial Asian population.

Matched MeSH terms: Pulmonary Veins/radiography*

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 Veins/abnormalities*

It is not asthma! An arcane case of 'Scimitar' syndrome: A case report

Ganapathi P, Nurul Yaqeen ME, Zamzurina AB

Med J Malaysia, 2020 03;75(2):184-185.

PMID: 32281607

'Scimitar' syndrome in adulthood is usually asymptomatic. Significant structural abnormalities symptoms usually manifest early during infancy or young childhood with features of congestive heart failure from significant shunting of the anomalous pulmonary venous drainage. Diagnosis of 'Scimitar' Syndrome in adults is rare and usually an incidental finding on chest radiograph. Here, we report a case of an adult who presented with symptoms in her 40's. This syndrome has never been reported nor discussed in Malaysia. This is the first case report of 'Scimitar' Syndrome in Malaysian literature. The diagnostic dilemma, medical management, and multi-disciplinary management by cardiology, physiotherapy and pulmonary rehabilitation teams are discussed.

Matched MeSH terms: Pulmonary Veins

Fulltext The blue man who presented with a stroke

Koh KC, Wong MH

Malays Fam Physician, 2011;6(1):29-31.

PMID: 25606217 MyJurnal

A 19-year-old man presented with sudden onset of right eye ptosis, diplopia and giddiness. He had no previous medical illnesses with negative history of exertional dyspnoea, epistaxis, haemoptysis, palpitations, chest pain and chronic cough. Examination revealed central cyanosis, digital clubbing, polycythaemia, partial ptosis of right eye, diplopia on right gaze and dilated right pupil. Examination of the chest revealed pectus excavatum but no cardiac murmurs were heard. Investigations revealed a solitary right pulmonary arteriovenous malformation with two feeder vessels which were successfully embolized surgically.

Matched MeSH terms: Pulmonary Veins

Progressive pulmonary vein stenosis in Down syndrome infant: a rare cause of pulmonary hypertension

Norazah Z, Joyce Darshinee DS

Med J Malaysia, 2018 04;73(2):119-120.

PMID: 29703879 MyJurnal

Pulmonary veins stenosis in a Down Syndrome infant with normal connection pulmonary vein is rare and precise incidence of this disease is unknown. We report a case of progressive multiple pulmonary vein stenosis in a Down Syndrome infant with congenital heart disease and transient myeloproliferative leukaemia. This case-report aims to improve awareness among physicians and sonographers of this disease and the importance of pulmonary vein assessment in congenital heart disease with unexplained pulmonary hypertension.

Matched MeSH terms: Pulmonary Veins

Single-stage open repair of severe asymmetric pectus excavatum and mitral valve replacement in connective tissue disease

Pau CP, Chong KS, Yakub MA, Khalil AA

Asian Cardiovasc Thorac Ann, 2021 May 04.

PMID: 33947231 DOI: 10.1177/02184923211014004

We present a 14-year-old boy with Loey-Dietz syndrome with severe mitral regurgitation, pectus excavatum and scoliosis. The Haller index was 25. The heart was displaced into the left hemithorax. The right inferior pulmonary vein was very close to the sternum and vertebral body. Single-stage surgery was performed. An osseo-myo-cutaneous pedicled flap was created by sterno-manubrial junction dislocation and rib resection with bilateral internal mammary arteries supplying the flap. Cardiopulmonary bypass and mitral valve replacement was performed. The defect was bridged with three straight plates. The flap was laid on top and anchored. Early outcome at three months was good.

Matched MeSH terms: Pulmonary Veins

Fulltext The rare congenital anomaly of scimitar syndrome: an unusual presentation in symptomatic adult

Siti Zulaiha Binti Che Hat

Malaysian Journal of Medicine and Health Sciences, 2020;16(3):334-336.

MyJurnal

Scimitar syndrome is a rare congenital heart defect occurring in 1 to 3 per 100,000 live births. This is a case of a 26 years old lady presenting with episodic fainting spells since the age of 18 years old. She was initially diagnosed with epilepsy until a referral to our centre found a soft splitting of the second heart sound and multiple premature ventricular complexes on ECG. The computed tomography of the pulmonary artery confirmed the diagnosis if Scim- itar syndrome in the presence of anomalous single right pulmonary vein draining into infra-diaphragmatic systemic venous circulation. A corrective open-heart surgery to re-implant the pulmonary vein was performed with excellent clinical outcomes. Therefore, it is crucial for clinicians to embody high index of suspicion of congenital anomaly even in adults presenting with indefinite clinical symptoms. This report also represents the first published case of adult Scimitar syndrome from Malaysia.

Matched MeSH terms: Pulmonary Veins

Fulltext Successful surgical strategy for ventral thoracic spinal perimedullary spinal arteriovenous fistulas: Case report

Subramaniam SM, Ishii K, Sheng CJ, Nakatomi H, Takai K, Saito N

Surg Neurol Int, 2019;10:251.

PMID: 31893152 DOI: 10.25259/SNI_516_2019

Background: Spinal arteriovenous fistulas (AVFs) are vascular lesions that often pose significant surgical challenges. This is particularly true for those located close to the anterior spinal artery. Here, we analyzed the surgical options for treating an anterior perimedullary AVF (pAVFs).

Case Description: A 66-year-old male with the right lower extremity weakness was diagnosed with a spinal dural AVF at the L1 level. It was initially treated with open surgery followed by CyberKnife radiosurgery at another institution. Five years later, he presented with a persistent pAVF fistula now involving the T11 level; the major feeder originated on the left at the T7-T8 level (e.g., involving a left-sided "duplicated" anterior spinal artery). Utilizing a three-dimensional (3D) computer tomography (CT) guided approach; he underwent a left-sided posterolateral T10-T12 laminectomy, sufficient to allow for 30-40° of anterior spinal cord rotation. This was performed under neurophysiological monitoring without any significant changes. Surgery included indocyanine green video angiography, temporary feeder clipping, and complete occlusion of the AVF, followed by complete clipping/resection as confirmed on postoperative magnetic resonance imaging.

Conclusion: Utilizing a 3D CT image, a ventral pulmonary arteriovenous malformation was excised utilizing a left-sided posterolateral approach allowing for 30-40° of cord rotation.

Matched MeSH terms: Pulmonary Veins

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