Succesfull management of a giant pulmonary artery aneurysm associated with severe congenital pulmonary valve stenosis in an adult patient

Pulmonary artery aneurysms are rare entities usually associated with congenital anomalies that increase blood flow/pressure in the pulmonary circulation, like left to right shunts and/or severe pulmonary valve pathologies. Clear re-commendations about their management are lacking. We report on a case of severe congenital pulmonary valve stenosis associated with a giant pulmonary artery aneurysm, secondary right ventricular outflow tract obstruction and right to left atrial shunt. We discuss the diagnostic pathway, therapeutic approach and patient evolution. The case report proves that a complete imaging of the heart and blood vessels is mandatory with severe pulmonary valve stenosis, in order to better describe its repercussions on the right ventricle and pulmonary circulation. They might be under detected by simple tran-sthoracic echocardiography but their presence has important therapeutic implications.

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P255 A fall from the tractor: a case report

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.116 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

S Habjan ◽

D Cantisani ◽

I S Scarfo` ◽

V Avitabile ◽

F Romeo ◽

...

Keyword(s):

Pulmonary Artery ◽

Transesophageal Echocardiography ◽

Pulmonary Valve ◽

Interventricular Septum ◽

Artery Aneurysm ◽

Left Pulmonary Artery ◽

Pulmonary Trunk ◽

Systolic Pulmonary Artery Pressure ◽

Pulmonary Artery Aneurysm ◽

The Right

Abstract Introduction Pulmonary artery aneurysms (PAAs) are rare and infrequently diagnosed clinical conditions. They can have congenital, acquired or idiopathic causes. PAAs can be asymptomatic or present with non-specific manifestations even at large diameters up to 70 mm. Rupture and dissection are the main life threating complications. The optimal treatment remains uncertain. There are no clear guidelines regarding the therapeutical approach. Case presentation A 78-year-old male presented at our medical facility to investigate further a pulmonary artery aneurysm discovered accidentally after a traumatic fall from a farmer tractor. He had no important chronic diseases, but in his medical history it was reported that he was excluded from the military service on top of a cardiac murmur, never investigated with an echocardiography exam. The fall from the tractor caused a fracture of the second and third costal ribs with hematoma and a commotional cranial trauma. At the thoraco-abdominal computed tomography (CT) scan conducted after the fall emerged an important fusiform dilatation of the principal pulmonary trunk and the left pulmonary artery (3.8 cm and 5 cm respectively). There was also a mild dilatation of the right pulmonary artery (3 cm), thickening of the pulmonary valve cusps and dilatation of the right cardiac chambers with flattening of the interventricular septum. Pulmonary embolism was excluded. Transthoracic and transesophageal echocardiography revealed a dysplastic pulmonary valve, with thickened cusps and moderate stenosis with insufficiency. The jet of the right ventricular outflow tract was eccentric with an anterolateral direction and a correlated dilatation of the common pulmonary trunk (3.8 cm) and the left pulmonary artery (5 cm). The right ventricle appeared not dilated and the tricuspid regurgitation was mild with a normal systolic pulmonary artery pressure. The dilated pulmonary artery did not interfere with the flow in the left main coronary artery. We concluded that the pulmonary artery aneurysm was mainly due to the congenital pulmonary valvulopathy. The patient underwent successful pulmonary trunk, left pulmonary artery and pulmonary valve replacement with a bioprosthetic valved conduit. Conclusion Congenital pulmonary valvulopathy can remain clinically silent lifelong and is commonly discovered during exams performed due to other clinical circumstances. The eccentric jet caused by the valvulopathy can be the reason for dilatation of the pulmonary trunk and pulmonary arteries. Transthoracic and transesophageal echocardiography are competent tools for clarifying the cause of the pulmonary artery aneurysm and therefore are essential in the decision making regarding the treatment strategy. Abstract P255 Figure. Transesophageal echocardiography images

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Partial anomalous pulmonary venous drainage repair concomitant with bilateral semilunar valve replacements and pulmonary artery reconstruction for an adult female 20 years after initial truncus arteriosus repair

Cardiology in the Young ◽

10.1017/s1047951117002736 ◽

2018 ◽

Vol 28 (3) ◽

pp. 514-515

Author(s):

Toshiyuki Iwaya ◽

Koichi Sughimoto ◽

Kagami Miyaji

Keyword(s):

Pulmonary Artery ◽

Pulmonary Valve ◽

Right Ventricular Outflow Tract ◽

Venous Drainage ◽

Ventricular Outflow Tract ◽

Anomalous Pulmonary Venous Drainage ◽

Right Pulmonary Artery ◽

Pulmonary Artery Reconstruction ◽

The Right ◽

Artery Reconstruction

AbstractWe describe the case of a 21-year-old patient who underwent repairs for multiple lesions including aortic and pulmonary valve replacements, right ventricular outflow tract reconstruction, revision of the right pulmonary artery route, and a repair of partial anomalous pulmonary venous drainage, which was diagnosed during this fourth sternotomy. For these patients with adult CHD, it is most important to address all underlying factors as much as possible at the redo surgery.

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Pulmonary valve stenosis causing massive pulmonary artery aneurysm

Journal of Cardiovascular Medicine ◽

10.2459/jcm.0b013e328337d83e ◽

2012 ◽

Vol 13 (9) ◽

pp. 593-596 ◽

Cited By ~ 2

Author(s):

Sameer Islam ◽

Cihan Cevik ◽

Ebtesam A. Islam ◽

Ekachai Singhatiraj ◽

Jason Jones ◽

...

Keyword(s):

Pulmonary Artery ◽

Pulmonary Valve ◽

Artery Aneurysm ◽

Pulmonary Artery Aneurysm ◽

Pulmonary Valve Stenosis

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A simplified technique for pulmonary artery aneurysm repair in a lung transplant recipient with right ventricular outflow tract obstruction

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/j.jtcvs.2012.08.027 ◽

2013 ◽

Vol 145 (1) ◽

pp. 295-296 ◽

Cited By ~ 2

Author(s):

Giorgio Zanotti ◽

Matthew G. Hartwig ◽

R. Duane Davis

Keyword(s):

Transplant Recipient ◽

Pulmonary Artery ◽

Right Ventricular ◽

Lung Transplant ◽

Artery Aneurysm ◽

Right Ventricular Outflow Tract ◽

Ventricular Outflow Tract ◽

Outflow Tract ◽

Pulmonary Artery Aneurysm ◽

Aneurysm Repair

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Restrictive right ventricular performance assessed by cardiac magnetic resonance after balloon valvuloplasty of critical pulmonary valve stenosis

Cardiology in the Young ◽

10.1017/s1047951115000724 ◽

2015 ◽

Vol 26 (3) ◽

pp. 556-568 ◽

Cited By ~ 1

Author(s):

Ikram Massoud ◽

Nader Botros ◽

Atef Yehia ◽

Hassan Abdelghafoor ◽

Mohamed Donya ◽

...

Keyword(s):

Late Gadolinium Enhancement ◽

Right Ventricular ◽

Pulmonary Valve ◽

Right Ventricular Outflow Tract ◽

Ventricular Outflow Tract ◽

Balloon Valvuloplasty ◽

Pulmonary Valve Stenosis ◽

Gadolinium Enhancement ◽

The Right

AbstractBackgroundLittle data are published about right ventricular diastolic performance in patients with critical pulmonary valve stenosis after balloon pulmonary valvuloplasty thus far.MethodsA total of 44 patients with isolated critical pulmonary valve stenosis who had undergone balloon valvuloplasty with haemodynamic recordings were enrolled to the study; 33 patients who came for follow-up underwent further imaging by echocardiography after 6 months and their right ventricular functional parameters were compared with 33 control patients of the same age and sex. Out of 33 patients, 21 underwent cardiac MRI with late gadolinium enhancement to assess the presence of right ventricular fibrosis.ResultsThe right ventricular systolic pressure (p<0.0001) and right ventricular outflow tract gradient (p<0.0001) decreased acutely (p<0.0001) after balloon valvuloplasty. During follow-up, M-mode left ventricular end diastolic dimension (p<0.001) and end systolic dimension increased (p<0.001), whereas right ventricular end diastolic dimension decreased (p<0.001). Compared with controls, patients (n=33) had significantly reduced tricuspid annular Ea and higher E/Ea (p<0.001). Right ventricular systolic dysfunction was also suggested by reduced tricuspid annular systolic velocity (p<0.001). Late gadolinium enhancement was demonstrated in 13 out of 21 patients with restrictive physiology, which involves the anterior right ventricular outflow tract, anterior wall, and inferior wall. The right ventricular late gadolinium enhancement score correlated positively with age (r=0.7, p<0.001) and right ventricular mass index (r=0.52, p<0.001).ConclusionThe persistence of right ventricular diastolic dysfunction after relief of chronic pressure overload of critical pulmonary valve stenosis suggests that a factor – other than increase in afterload – is involved in this physiology. Fibrosis is the most likely factor responsible for persistence of restrictive physiology as documented by late gadolinium enhancement.

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