scholarly journals A Case of Isolated Bicuspid Pulmonic Valve and Pulmonary Artery Aneurysm

Author(s):  
Mohammad Sahebjam ◽  
Neda Toofaninejad

A 33-year-old woman with a history of thyroid surgery for thyroid cancer and radioactive iodine therapy was referred for echocardiography due to dyspnea on exertion. Transthoracic echocardiography showed normal left ventricular size and function (the ejection fraction = 55%), a prolapsing mitral valve with redundant chordae, mild mitral regurgitation, a tricuspid aortic valve, mild aortic insufficiency, and mild tricuspid regurgitation. The most remarkable echocardiographic findings were moderate right ventricular dilation with mild systolic dysfunction, moderate right atrial dilation, an aneurysmal pulmonary artery (the main pulmonary artery = 47 mm), mild pulmonary stenosis (the peak gradient = 22 mmHg), and severe pulmonary regurgitation (the vena contracta = 6–7 mm and the pressure half time = 105 ms). Transesophageal echocardiography with the use of 3D modalities demonstrated a bicuspid pulmonic valve with doming and poor coaptation of the pulmonic valve leaflets (Figure 1). Additionally, a large patent foramen ovale was visualized in color Doppler (the flap separation = 2 mm and the tunnel length = 11 mm) with bubble passage in agitated saline injection. Bicuspid pulmonic valves constitute a rare finding, and they are most often associated with other congenital heart diseases. Isolated bicuspid pulmonic valves are extremely rare, with an incidence rate of about 0.1% in clinical practice.1 Pulmonary artery aneurysms also comprise a rare abnormality, with an incidence rate of approximately 1 in 14 000 cases in most studies.2 The association between bicuspid pulmonic valves and pulmonary artery aneurysms has been reported, and the pathophysiologic causes of this association include hemodynamic alterations due to bicuspid pulmonic valves and most likely the abnormal migration of neural crest cells.3  The diagnosis of a bicuspid pulmonic valve by 2D imaging is challenging and sometimes impossible. Using 3D echocardiography and reconstruction confers a better assessment of the pulmonic valve morphology and identification of bicuspid pulmonic valves.

2021 ◽  
pp. 1-6
Author(s):  
Ergin Arslanoglu ◽  
Kenan Abdurrahman Kara ◽  
Fatih Yigit ◽  
Cüneyt Arkan ◽  
Esra Ozcan ◽  
...  

Abstract Pulmonary artery aneurysms are rare. They are characterised by an aneurysmatic dilatation of the pulmonary vascular bed, including the main pulmonary artery or the accompanying pulmonary artery branches. Increases in pulmonary flow and pulmonary artery pressure increase the risk of rupture: when these conditions are detected, surgical intervention is necessary. This study is a retrospective analysis of 33 patients treated in our paediatric cardiac surgery clinic from 2012 to 2020. Aneurysms and pseudoaneurysms in patients who were patched for right ventricular outflow tract reconstruction and corrected with a conduit were excluded from the study. Seventeen (51.5%) of the patients included in the study were female and 16 (48.5%) were male. The patients were aged between 23 and 61 years (mean 30.66 ± 12.72 years). Graft interpositions were performed in 10 patients (30.3%) and pulmonary artery plications were performed in 23 patients (69.7%) to repair aneurysms. There was no significant difference in mortality between the two groups (p > 0.05). Pulmonary artery aneurysm interventions are safe, life-saving treatments that prevent fatal complications such as ruptures, but at present there is no clear guidance regarding surgical timing or treatment strategies. Pulmonary artery interventions should be performed in symptomatic patients with dilations ≥5 cm or asymptomatic patients with dilations ≥8 cm; pulmonary artery pressure, right ventricular systolic pressure, and pulmonary artery aneurysm diameter must be considered when planning surgeries, their timing, and making decisions regarding indications. Experienced surgical teams can achieve satisfactory results using one of the following surgical techniques: reduction pulmonary arterioplasty, plication, or graft replacement.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
K Sakata ◽  
H Mitsuda ◽  
J Ito ◽  
A Isaka ◽  
M Furuya ◽  
...  

Abstract Background Pulmonary artery aneurysm (PAA) occurs in some patients with pulmonary arterial hypertension (PAH) and can be accompanied by various complications. Objective The aim of the this study is to evaluate the characteristics of cardiac dysfunction and the prognostic impact of PAA by analysing the outcome of patients with PAH complicated by PAA. Methods We performed echocardiography and right-heart catheterization in 130 PAH patients. We measured the maximum dimensions of the main pulmonary artery (MPA) trunk. We evaluated the right ventricular (RV) function and the five-year prognosis of patients with PAA. Results The maximum MPA trunk diameter of >40 mm by echocardiography was defined as PAA. PAA appeared in 32 of the 130 PAH patients (24.6%: Group PAA), other 98 patients were defined as Group non-PAA. Systolic pulmonary artery pressure (76 ±18 vs. 61 ± 21 mmHg, P = 0.0008) and mean right atrial pressure (10.0 ± 8.1 vs. 5.4 ± 3.8 mmHg, P < 0.0001) were significantly higher in Group PAA than in Group non-PAA. RV end-diastolic area index was significantly larger (37.3 ± 8.8 vs. 32.3 ± 7.8 mmHg, P = 0.0048), and RV fractional area change (32 ± 8 vs. 36 ± 9 %, P = 0.0176) and RV longitudinal strain amplitude (-16 ±5 vs. -20 ± 6, P = 0.0017) were significantly lower in Group PAA than those in Group non-PAA. During the five-year follow-up period, 20 patients (15%) died. The five-year mortality rate was significantly higher in Group PAA compared to Group non-PAA (30% vs. 10%, P = 0.007). Conclusion In PAH patients with PAA, RV dysfunction was more severe and the prognosis was poor. Assessment of PAA can be a useful index for the extraction of high-risk PAH patients.


2002 ◽  
Vol 10 (2) ◽  
pp. 167-169 ◽  
Author(s):  
Saket Agarwal ◽  
Ujjwal Kumar Chowdhury ◽  
Anita Saxena ◽  
Ruma Ray ◽  
Sanjeev Sharma ◽  
...  

Aneurysm formation of the main pulmonary artery is rare. Its natural history is not well understood and there are no clear guidelines on optimal treatment. A 20-year-old woman with a huge saccular aneurysm of the main pulmonary artery, underwent repair with a pericardial patch and concomitant reconstruction of the pulmonary valve. The patient was doing well on follow-up at 6 months; echocardiography revealed a good repair with mild to moderate pulmonary regurgitation.


2019 ◽  
Vol 30 (1) ◽  
pp. 159-160
Author(s):  
Davide Carino ◽  
Nikita Mehta ◽  
Alejandro Fernández-Cisneros ◽  
Daniel Pereda

Abstract Pulmonary artery aneurysms are rare but are associated with a significant risk of rupture and dissection. Moreover, pulmonary valve regurgitation and/or stenosis often coexist. In this study, we present a case of a pulmonary artery aneurysm with severe pulmonary regurgitation in a patient with pulmonary hypertension treated with aneurysm resection and pulmonary valve repair.


2017 ◽  
Vol 5 (21) ◽  
pp. 32
Author(s):  
Audra Schwalk ◽  
Gilbert Berdine

Pulmonary artery (PA) aneurysms are uncommon and often diagnosed post-mortem. Theyare characterized by a PA/aorta diameter ratio greater than 2 on transthoracic echocardiographyor a pulmonary artery diameter greater than 4 or 5 cm on computed tomography. The mostcommon conditions associated with pulmonary artery aneurysm are congenital heart defectswith left-to-right shunts and pulmonic valve abnormalities. There are also numerous causesof acquired pulmonary artery aneurysms, including infection, vasculitis, pulmonary arterialhypertension, trauma, neoplasm and pulmonary embolism. Symptoms of PA aneurysm areusually non-specific, and physical examination findings are variable depending on the underlyingcause. Work-up includes various imaging modalities, transthoracic echocardiography, and rightheart catheterization. The gold standard treatment is surgery, but in select patients, conservativemanagement with close monitoring can be pursued.


Author(s):  
Sophia Alexis ◽  
Ismail El-Hamamsy ◽  
Michael Robbins

Background Bicuspid pulmonic valves are quite uncommon, being described in only 0.1% of donor hearts, while pulmonary artery aneurysms are even more rare, having been found in 8 out of 109,571 autopsies. This rarity makes it difficult to characterize the relationship between them. Materials & Methods We describe the case of a 66-year-old female who was found to have a bicuspid pulmonic valve and pulmonary artery aneurysm (5.1cm) on imaging by her cardiologist. Discussion & Conclusion This case raises the question of whether the association between bicuspid semilunar valve disease and vascular wall anomalies are more genetic or hemodynamic. Even on the aortic side, despite the robust association between bicuspid aortic valves and thoracic aortic aneurysms, the mechanism still remains unclear. In our patient there was no significant gradient across the bicuspid pulmonic valve, suggesting that hemodynamics are not the primum mobile of this association.


Author(s):  
Srujan Ganta ◽  
John Artrip ◽  
Eleanor L. Schuchardt ◽  
Wyman Lai ◽  
Justin Ryan ◽  
...  

We describe the management of an infant presenting with severe heart failure at 6 weeks of age found to have an anomalous single coronary artery originating from the main pulmonary artery (MPA). This patient was transferred to our hospital and ultimately had their coronary artery translocated to the ascending aorta successfully. Preoperative severe left ventricular (LV) dysfunction and moderate/severe mitral regurgitation (MR) improved to normal function and mild-to-moderate MR 6 weeks postrepair. Three-dimensional CT reconstructions proved valuable and allowed for accurate preoperative planning leading to successful coronary transfer.


1989 ◽  
Vol 19 (2) ◽  
pp. 343
Author(s):  
Hong Lyeol Lee ◽  
Myong Ki Hong ◽  
Kyung Kwon Paik ◽  
Seung Jae Tahk ◽  
Woong Ku Lee ◽  
...  

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Emily K Zern ◽  
Paula Rambarat ◽  
Samantha Paniagua ◽  
Elizabeth Liu ◽  
Jenna McNeill ◽  
...  

Introduction: The pulmonary artery pulsatility index (PAPi), calculated from the ratio of pulmonary artery pulse pressure to right atrial pressure, was initially described as a novel predictor of right ventricular failure after inferior myocardial infarction or left ventricular assist device implantation. Whether PAPi is associated with adverse outcomes in broader samples is unknown. Hypothesis: A lower PAPi is associated with mortality in a broad population referred for right heart catheterization. Methods: We examined consecutive patients undergoing right heart catheterization between 2005-2016 in a hospital-based cohort. The following exclusion criteria were applied: shock or cardiac arrest within 24 hours of catheterization, presence of mechanical circulatory support, prior cardiac transplant, prior valvular surgery, or those with missing key clinical covariates. Multivariable Cox models were utilized to examine the association between PAPi and mortality. Analyses were adjusted for age, sex, BMI, hypertension, diabetes, prior myocardial infarction, and prior heart failure. Results: We studied 8559 patients with mean age 63 years and 40% women. We found that patients in the lowest quartile of PAPi were younger, with greater proportion of men, and higher BMI, yet similar NT-proBNP compared with other quartiles ( Table 1 ). Over 12.5 years of follow-up, there were 2441 death events. Patients in the lowest PAPi quartile had a 31% greater risk of death compared with the highest quartile (multivariable adjusted HR 1.31, 95% CI 1.15-1.48, p<0.001), whereas no differences in survival were seen among individuals in quartile 2 or 3 (p>0.05 vs quartile 4 for both). Conclusions: Patients in the lowest PAPi quartile had a 31% increased risk of all-cause mortality in a broad population referred for right heart catheterization. These findings highlight a potential role for PAPi in identifying high-risk individuals across a spectrum of disease.


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