Prenatal diagnosis of a distal aortopulmonary window with holosystolic pulmonary regurgitation

Author(s):  
N.M. Normuradova

The case of pulmonary artery regurgitation and through the systole and early ventricular diastole in the pulmonary valve, due to the distal aorto-pulmonary window on the fetus of 21+4 weeks of gestation is presented. Cardiomegaly due to the right heart and pronounced tricuspid regurgitation were also noted.

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Matteo Bellettini ◽  
Antonio Montefusco ◽  
Andrea Angelini ◽  
Fulvio Orzan ◽  
Fabrizio D’Ascenzo ◽  
...  

Abstract Methods and results A 70-year-old woman presented to our outpatient clinic complaining of worsening dyspnoea in the last 3 months. She had a medical history of hypertension, diabetes, dyslipidemia, and paroxysmal atrial fibrillation. We performed a comprehensive evaluation starting with a transthoracic echocardiogram that showed a dilatation of right ventricle with normal function, severe pulmonary regurgitation, and moderate tricuspid regurgitation with estimated pulmonary artery systolic pressure of 55 mmHg; the left ventricle had normal dimension and function, with mild aortic and mitral regurgitation, and a subaortic membrane which caused a mild obstruction (maximum gradient 17 mmHg). The cardiac magnetic resonance (CMR) confirmed the enlargement of the right ventricle and of the pulmonary artery trunk (51 mm) and the severity of pulmonary regurgitation (regurgitant fraction of 41%). CMR also clearly showed the VSD just below the subaortic membrane and the left to right shunt with a jet that appeared to proceed straight from the left ventricle through the pulmonary valve (Figure 1A). The estimated Qp/Qs was 1.6 and no intramyocardial late enhancement was present. Pulmonary pressures and pulmonary vascular resistance were normal at the right heart catheterization and the Qp/Qs ratio calculated invasively was 1.45. Considering patient high-risk profile for coronary artery disease, a coronary angiography was also performed showing an abnormal origin of the right coronary artery (RCA) from the mid-portion of the left anterior descending coronary artery (LAD) with two significant stenosis: one involving the bifurcation of RCA and the other the mid-portion of the LAD (Figure 1B). The coronary computed tomography angiography (CCTA) showed a benign course of the RCA anterior to the pulmonary artery towards the auriculoventricular groove (Figure 1C, D). Taking into account all these findings, multidisciplinary heart team decided to perform a cardiac surgery intervention of pulmonary valve and trunk replacement, closure of ventricular septal defect and two coronary bypass grafts on LAD and RCA. Conclusions This case represents a combination of some rare congenital heart abnormalities where multimodality cardiovascular imaging techniques were essential to establish a proper diagnosis and to plan an adequate surgical repair. We hypothesize that the peculiar orientation of the VSD jet may have caused the pulmonary trunk dilatation considering that neither the shunt, nor the pulmonary pressure appear to have been of sufficient magnitude to cause it. Pulmonary ectasia and the damage inflicted by the jet to the cusps of the valve have led to the severe valvular insufficiency. While aortic and tricuspid regurgitation are known to be associated with VSD, to the best of our knowledge this is the first report of pulmonary regurgitation secondary to VSD.


Author(s):  
Tiffany A. Camp ◽  
Stephanie Hequembourg ◽  
Richard S. Figliola ◽  
Tim McQuinn

The operating pressures in the right heart are significantly lower than those of the left heart and with marked differences in the circulation impedances. The pulmonary circulation shows a tolerance for mild regurgitation and pressure gradient [1]. Pulmonary regurgitation fractions on the order of 20% and transvalvular pressure gradients of less than 25mm Hg are considered mild [4]. Given this tolerance, we examine the concept of using a motionless valve to regulate flow in the pulmonary position. In a previous study, the use of fluid diodes was shown to be a promising concept for use as a pulmonary valve [2]. In this study, we test two different diode designs. For each diode valve, flow performance was documented as a function of pulmonary vascular resistance (PVR) and compliance. Tests were done using a pulmonary mock circulatory system [3] over the normal adult range of PVR and compliance settings.


2013 ◽  
Vol 34 (suppl 1) ◽  
pp. P310-P310
Author(s):  
P. De Meester ◽  
A. Van De Bruaene ◽  
P. Herijgers ◽  
J.- U. Voigt ◽  
M. Delcroix ◽  
...  

2013 ◽  
Vol 168 (4) ◽  
pp. 3866-3871 ◽  
Author(s):  
Pieter De Meester ◽  
Alexander Van De Bruaene ◽  
Paul Herijgers ◽  
Jens-Uwe Voigt ◽  
Marion Delcroix ◽  
...  

Author(s):  
Tiffany Camp ◽  
Richard Figliola ◽  
Tim McQuinn ◽  
T.-Y. Hsia ◽  
Tim Conover

The operating pressures in the right heart are significantly lower than those of the left heart and with marked differences in the circulation impedances. The pulmonary circulation shows a tolerance for mild regurgitation and pressure gradient [1]. Pulmonary regurgitation fractions on the order of 20% and transvalvular pressure gradients of less than 25mm Hg are considered mild [2]. Given this tolerance, we examine the concept of using a motionless valve to regulate flow in the pulmonary position. In a previous study, the use of fluid diodes was shown to be a promising concept for use as a pulmonary valve [3]. In this study, we test a fluid diode both in a bench top circulatory system [4] and in a swine model and compare the pressure gradients and regurgitant fractions.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
E Dobrzanska ◽  
M Tomaszewski ◽  
R Zarczuk ◽  
A Tomaszewski ◽  
E Czekajska-Chehab

Abstract A 38y. old woman was admitted to a Cardiology Department due to increased exertional dyspnea and decreased exercise tolerance. Echocardiography performed in an outpatient setting has found a substantial enlargement of the right ventricle and severe tricuspid regurgitation. Physical examination significantly enlarged liver, pulsation of jugular veins and numerous scars in the pits elbow. ECG sinus rhythm 85 / min. Right axis deviation. LPH. Hypertrophy of the right atrium. QS in V1-V4. Transthoracic and Transesophageal Echocardiography (TTE,TEE) EF 64%, a significant increase in a right heart chambers (RVDD 4.1 cm, severe tricuspid regurgitation with completely disappearing of tricuspid valve ( only part of septal leaflet was present, which was a consequence of pressure equalization between the right atrium and the right ventricle). In addition, it revealed the structure connected with the pulmonary valve leaflet and moving between the right ventricular outflow tract and pulmonary trunk (most probably healed vegetation, 1.2 x 0.5 cm ). Computed tomography (CT) confirmed the significant enlargement of right heart chambers (EDV 335 ml, ESV 143 ml, SV 192 ml, EF ∼ 58%) with displacement of interatrial septum to the left and the flattening of the interventricular septum . Complete destruction of the tricuspid valve leaflets, with the remaining residual part of septal leaflet was observed. The pulmonary valve was connected mobile irregular structure 2,5 cm x 0,5 cm. Laboratory tests revealed a history of cytomegalovirus infection (p / body IgG> 500,000U / ml). Other tests (HIV, hepatitis B, reaction W-R) - were negative. There was no laboratory and clinical signs of active infection at present. Patient demanded to be discharged from the hospital and refused operation. DISCUSSION Echocardiography did not confirm diagnosis of pulmonary hypertension. D-dimer values of 396 ng / ml (normal <500 ng / ml) excluded suspicion of pulmonary embolism. Left ventricular ejection fraction was normal (EF ∼ 64%), BNP reached the value of 153 pg / ml (normal 0-100pg / ml). This case deserves attention because it documents severe right heart endocarditis by the person using drugs intravenously with an extremely rare takeover of both right heart valves and septic pulmonary embolism. Despite such a large morphological change in the heart of a patient remains in a relatively good clinical condition (NYHA class II/ III). The observed structure of the pulmonary trunk should be considered as healed vegetation. In the absence of consent to the surgery the patient is still treated pharmacologically. Abstract P855 Figure. Pic.1


Author(s):  
Isaac Wamala ◽  
Christopher J. Payne ◽  
Mossab Y. Saeed ◽  
Daniel Bautista-Salinas ◽  
David Van Story ◽  
...  

Abstract Purpose In clinical practice, many patients with right heart failure (RHF) have elevated pulmonary artery pressures and increased afterload on the right ventricle (RV). In this study, we evaluated the feasibility of RV augmentation using a soft robotic right ventricular assist device (SRVAD), in cases of increased RV afterload. Methods In nine Yorkshire swine of 65–80 kg, a pulmonary artery band was placed to cause RHF and maintained in place to simulate an ongoing elevated afterload on the RV. The SRVAD was actuated in synchrony with the ventricle to augment native RV output for up to one hour. Hemodynamic parameters during SRVAD actuation were compared to baseline and RHF levels. Results Median RV cardiac index (CI) was 1.43 (IQR, 1.37–1.80) L/min/m2 and 1.26 (IQR 1.05–1.57) L/min/m2 at first and second baseline. Upon PA banding RV CI fell to a median of 0.79 (IQR 0.63–1.04) L/min/m2. Device actuation improved RV CI to a median of 0.87 (IQR 0.78–1.01), 0.85 (IQR 0.64–1.59) and 1.11 (IQR 0.67–1.48) L/min/m2 at 5 min (p = 0.114), 30 min (p = 0.013) and 60 (p = 0.033) minutes respectively. Statistical GEE analysis showed that lower grade of tricuspid regurgitation at time of RHF (p = 0.046), a lower diastolic pressure at RHF (p = 0.019) and lower mean arterial pressure at RHF (p = 0.024) were significantly associated with higher SRVAD effectiveness. Conclusions Short-term augmentation of RV function using SRVAD is feasible even in cases of elevated RV afterload. Moderate or severe tricuspid regurgitation were associated with reduced device effectiveness.


2021 ◽  
Vol 49 (1) ◽  
pp. 030006052098465
Author(s):  
Mingyue Cui ◽  
Binfeng Xia ◽  
Heru Wang ◽  
Haihui Liu ◽  
Xia Yin

Aortopulmonary window is a rare congenital heart disease that can increase pulmonary vascular resistance, exacerbate left-to-right shunt and lead to heart failure and respiratory tract infections. Most patients die during childhood. We report a 53-year-old male patient with a large aortopulmonary window combined with anomalous origin of the right pulmonary artery from the aorta, with Eisenmenger syndrome and without surgery.


2021 ◽  
pp. 1-3
Author(s):  
Claire Bertail-Galoin

Abstract A fistula between the pulmonary artery and the left atrium is a rare entity and its diagnosis is uncommon in the neonatal period. There are more reported surgical treatments in the literature than with a transcatheter closure. We report the case of a prenatal diagnosis of a large fistula between the right pulmonary artery and the left atrium with successful transcatheter closure with an Amplatzer duct occluder II 6/4 mm.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Atsuko Kato ◽  
Christian Drolet ◽  
Shi-Joon Yoo ◽  
Andrew Redington ◽  
Lars Grosse-Wortmann

Introduction: The left pulmonary artery (LPA) contributes more than the right (RPA) to total pulmonary regurgitation (PR) in patients after tetralogy of Fallot (TOF) repair, but the mechanism of this difference is not well known. We hypothesized that unilaterally increased pulmonary vascular resistance (PVR), resulting from lung compression by the enlarged and levorotated heart leads to greater PR in the LPA. This study aimed to analyze the interplay between heart and lung size, mediastinal geometry, and differential PR. Methods: This is a single-center retrospective analysis of 50 magnetic resonance studies in patients after TOF repair. Patients with more than mild discrete branch pulmonary artery stenosis were excluded. Blood flow was measured by phase-contrast velocity encoding within the branch pulmonary arteries. On the axial image with the largest total cardiac surface area, cardiac angle (α) between the thoracic anterior-posterior line and the interventricular septum, right and left lung areas as well as right and left hemithorax areas were measured (Figure). Results: There was no difference in LPA and RPA diameters. The LPA showed significantly less total forward flow (p=0.04), smaller net forward flow (p=<0.001), and greater RF (p=0.001) than the RPA. Left lung area was smaller than the right (p<0.001). RVEDVi correlated with LPA RF (R=0.48, p<0.001), but not with RPA RF. Larger RVEDVi correlated with a larger α angle (R=0.46, p<0.001), i.e. a more leftward cardiac axis and with smaller left lung area (R=-0.58, p<0.001). LPA RF, but not RPA RF, correlated inversely with left lung area indexed to the left hemithorax area (R=-0.34, p=0.02). Conclusions: An enlarged and levorotated heart - as a result of PR - is associated with smaller left lung size, and augments diastolic flow reversal in the LPA, presumably via increased left PVR. By imposing a further volume load on the RV, LPA regurgitation may thus close a positive feed-back loop of PR and RV dilatation.


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