Diagnosis and natural history of isolated congenital pulmonary regurgitation in fetal life

2000 ◽  
Vol 10 (2) ◽  
pp. 162-165 ◽  
Author(s):  
Kazuhiro Mori ◽  
Yasunobu Hayabuchi ◽  
Yasuhiro Kuroda

AbstractWe describe a rare instance of isolated pulmonary regurgitation caused by a dysplastic pulmonary valve which was detected prenatally. Fetal echocardiography demonstrated severe pulmonary regurgitation, and progressive cardiomegaly because of right ventricular volume overload. After birth, conservative therapy was successful in alleviating the pulmonary vascular resistance, and the pulmonary regurgitation gradually decreased.

2020 ◽  
Vol 30 (5) ◽  
pp. 737-739
Author(s):  
Omar Abu-Anza ◽  
Kaitlin Carr ◽  
Osamah Aldoss

AbstractWe report a case of a 15-year-old female who underwent combined hybrid pulmonary valve replacement and transcatheter atrial septal defect device closure, which was performed due to severe volume overload of the right side of the heart secondary to pulmonary regurgitation and atrial septal defect.


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Lars Søndergaard ◽  
Niels G Vejlstrup ◽  
Simone Theilade ◽  
Jens C Nilsson ◽  
Younes Boudjemline ◽  
...  

Background Despite successful repair of tetralogy of Fallot (TOF), the life expectancy for these patients remains lower than in the general population. This is mainly due to free pulmonary regurgitation (PR). The importance of maintaining a competent valve has been emphasized, but the optimal timing for intervention remains to be established. The purposes of this study were in a pig model to investigate the consequences of the duration of free PR on RV, and the reversibility of these changes after percutaneous pulmonary valve replacement. Methods Thirtysix farm pigs were divided into four groups (A, B, C and D) of nine pigs. At baseline group A, B and C had a percutaneous stent inserted into the pulmonary annulus to create free PR. After 4, 8 and 12 weeks, respectively, group A, B and C underwent percutaneous valve replacement. In order to allow remodelling of RV, the pigs were observed for four weeks after valve insertion before euthanasia. To examine RV function, MRI was performed before stent insertion, before valve replacement, and before euthanasia. Group D served as controls and underwent five MRI examinations: at baseline as well as after four, eight, twelve and sixteen weeks. Results When comparing the group D with group A, B & C, RVEDV and RVESV were found to gradually increase over time. Furthermore, these volumes normalised after valve replacement in group A and B, but remains increased in the group C. Similar, RVEF was normal after valve replacement in group A and B, but impaired in the group the long-term free PR compared to the controls. Conclusion Better timing of valve replacement in patients with free PR after TOF repair is of outermost clinical relevance. This study examined the impact of free PR on RV in a pig model. Despite the relative short duration of the volume overload, the RV underwent irreversible changes. This may be explained by the fact that, in contrast to TOF, the RV is not hypertrophic. The study showed that RV tolerates volume overload from free PR for a certain time, but too late intervention will cause irreversible deterioration of the ventricular function. Thus, the model may serve to identify predictors, e.g. tissue-Doppler measurements or natriuretic peptides, for RV function after valve replacement and thereby optimise timing of intervention.


Children ◽  
2021 ◽  
Vol 8 (3) ◽  
pp. 190
Author(s):  
Taehong Kim ◽  
Hoon Ko ◽  
Joung-Hee Byun ◽  
Hyoung Doo Lee ◽  
Hyungtae Kim ◽  
...  

Uhl’s anomaly is a very rare malformation of unknown cause, characterized by complete or partial absence of the right ventricular myocardium. The cardiac malformation causes progressive right heart failure, increased right-sided cardiac pressure, massive peripheral edema, and ascites. Patients usually present in infancy and rarely survive to adulthood. Previously, diagnosis was made at post-mortem evaluation, but advances in cardiac imaging now permit diagnosis during fetal life. We report a case of Uhl’s anomaly in a newborn baby imaged at 23 + 3 weeks of gestation by fetal echocardiography. There was an aneurysmally dilated thin-walled right ventricle with hypertrophy of the right ventricular apical muscles, the tricuspid valve was dysplastic, and the pulmonary valve leaflets were absent.


2021 ◽  
Vol 8 (7) ◽  
pp. 78
Author(s):  
Gabriele Egidy Assenza ◽  
Luca Spinardi ◽  
Elisabetta Mariucci ◽  
Anna Balducci ◽  
Luca Ragni ◽  
...  

Transcatheter closure of patent foramen ovale (PFO) and secundum type atrial septal defect (ASD) are common transcatheter procedures. Although they share many technical details, these procedures are targeting two different clinical indications. PFO closure is usually considered to prevent recurrent embolic stroke/systemic arterial embolization, ASD closure is indicated in patients with large left-to-right shunt, right ventricular volume overload, and normal pulmonary vascular resistance. Multimodality imaging plays a key role for patient selection, periprocedural monitoring, and follow-up surveillance. In addition to routine cardiovascular examinations, advanced neuroimaging studies, transcranial-Doppler, and interventional transesophageal echocardiography/intracardiac echocardiography are now increasingly used to deliver safely and effectively such procedures. Long-standing collaboration between interventional cardiologist, neuroradiologist, and cardiac imager is essential and it requires a standardized approach to image acquisition and interpretation. Periprocedural monitoring should be performed by experienced operators with deep understanding of technical details of transcatheter intervention. This review summarizes the specific role of different imaging modalities for PFO and ASD transcatheter closure, describing important pre-procedural and intra-procedural details and providing examples of procedural pitfall and complications.


Author(s):  
Jurate Bidviene ◽  
Denisa Muraru ◽  
Francesco Maffessanti ◽  
Egle Ereminiene ◽  
Attila Kovács ◽  
...  

AbstractOur aim was to assess the regional right ventricular (RV) shape changes in pressure and volume overload conditions and their relations with RV function and mechanics. The end-diastolic and end-systolic RV endocardial surfaces were analyzed with three-dimensional echocardiography (3DE) in 33 patients with RV volume overload (rToF), 31 patients with RV pressure overload (PH), and 60 controls. The mean curvature of the RV inflow (RVIT) and outflow (RVOT) tracts, RV apex and body (both divided into free wall (FW) and septum) were measured. Zero curvature defined a flat surface, whereas positive or negative curvature indicated convexity or concavity, respectively. The longitudinal and radial RV wall motions were also obtained. rToF and PH patients had flatter FW (body and apex) and RVIT, more convex interventricular septum (body and apex) and RVOT than controls. rToF demonstrated a less bulging interventricular septum at end-systole than PH patients, resulting in a more convex shape of the RVFW (r = − 0.701, p < 0.0001), and worse RV longitudinal contraction (r = − 0.397, p = 0.02). PH patients showed flatter RVFW apex at end-systole compared to rToF (p < 0.01). In both groups, a flatter RVFW apex was associated with worse radial RV contraction (r = 0.362 in rToF, r = 0.482 in PH at end-diastole, and r = 0.555 in rToF, r = 0.379 in PH at end-systole, respectively). In PH group, the impairment of radial contraction was also related to flatter RVIT (r = 0.407) and more convex RVOT (r = − 0.525) at end-systole (p < 0.05). In conclusion, different loading conditions are associated to specific RV curvature changes, that are related to longitudinal and radial RV dysfunction.


2019 ◽  
Vol 10 (2) ◽  
pp. 197-205 ◽  
Author(s):  
Chi Sum Yuen ◽  
Kwok Fai Lucius Lee ◽  
Inderjeet Bhatia ◽  
Nicholson Yam ◽  
Barnabe Antonio Rocha ◽  
...  

Background: Postcongenital heart surgery pulmonary regurgitation requires subsequent pulmonary valve replacement. We sought to compare the outcomes of pulmonary valve replacement after using bioprosthetic valves, porcine versus pericardial bioprosthesis. Method: Retrospective single-center study of consecutive pulmonary valve replacement in patients with pulmonary regurgitation following initial congenital cardiac surgery. From 2004 to 2016, 82 adult patients (53 males, 29 females) underwent pulmonary valve replacement at a mean age of 28.7 ± 8 years (range 18-52 years) with a mean time to pulmonary valve replacement of 24 ± 7 years (range 13-43 years). Porcine bioprosthetic valves (group 1, n = 32) and pericardial valves (group 2, n = 50) were used. Cardiac magnetic resonance imaging was performed (n = 54) at a mean of 18 ± 13 months before and 24 ± 21 months after pulmonary valve replacement. Results: No significant difference was seen between the groups except that the mean follow-up was longer for group 1 (5.02 ± 2.06 vs 4.08 ± 3.21 years). In-hospital mortality was 1.1%. Follow-up completeness was 100% with no late death. Mean right ventricular end-systolic and end-diastolic volumes reduced significantly in both the groups ( P < .001), whereas right ventricular ejection fraction remained unchanged (group 1, P = .129; group 2, P = .675) . Only the left ventricular end-diastolic volume increased in both the groups, but the increase was significant for group 2 only (group 1, P = .070; group 2, P = .015), whereas the left ventricular end-systolic and ejection fraction remained unchanged in both the groups. There was no reoperation for pulmonary valve replacement. Freedom from intervention was 93.8% (group 1) and 100% (group 2) at eight years after pulmonary valve replacement ( P = .407). Conclusion: Midterm outcomes of pulmonary valve replacement in our adult cohort were satisfactory. Both types of bioprosthetic valves performed comparably for eight years and were a good option in adults.


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