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Cardiology in the Young Feb 2024Double-chambered right ventricle is a rare and progressive condition that is characterised by obstruction of the right ventricular tract. Double-chambered right...
BACKGROUND/AIM
Double-chambered right ventricle is a rare and progressive condition that is characterised by obstruction of the right ventricular tract. Double-chambered right ventricle is usually associated with ventricular septal defect. Early surgical intervention is recommended in patients with these defects. Based on this background, the present study aimed to review early and midterm outcomes of primary repair after double-chambered right ventricle.
METHODS
Between January 2014 and June 2021, 64 patients with a mean age of 13.42 ± 12.31 years underwent surgical repair for double-chambered right ventricle. The clinical outcomes of these patients were reviewed and assessed retrospectively.
RESULTS
An associated ventricular septal defect was present in all the recruited patients; 48 (75%) patients of sub-arterial type, 15 (23.4%) of perimembranous, and 1 (1.6%) patient of muscular type. The patients were followed up for a mean period of 46.73 ± 27.37 months. During their follow-up, a significant decrease in the mean pressure gradient from 62.33 ± 5.52 mmHg preoperatively to 15.73 ± 2.94 mmHg postoperatively was observed (p < 0.001). Notably, there were no hospital deaths.
CONCLUSIONS
The development of double-chambered right ventricle in association with ventricular septal defect results in an increased pressure gradient within the right ventricle. The defect needs correction in a timely manner. In our experience, the surgical correction of double-chambered right ventricle is safe and shows excellent early and mid-term results.
Topics: Humans; Infant; Child, Preschool; Child; Adolescent; Young Adult; Adult; Heart Ventricles; Retrospective Studies; Heart Septal Defects, Ventricular; Time Factors; Arteries
PubMed: 37318004
DOI: 10.1017/S1047951123001531 -
The Journal of Thoracic and... Sep 2023
Topics: Humans; Tricuspid Valve; Heart Ventricles; Tricuspid Valve Insufficiency
PubMed: 34799091
DOI: 10.1016/j.jtcvs.2021.11.002 -
The Journal of Thoracic and... Oct 2023
Topics: Humans; Heart Ventricles; Vascular Surgical Procedures; Coronary Artery Disease; Coronary Circulation
PubMed: 37156361
DOI: 10.1016/j.jtcvs.2023.04.046 -
Heart, Lung & Circulation Sep 2019Pulmonary hypertension is a progressive and often fatal disease that frequently presents with dyspnoea on exertion and results in increased right ventricular afterload...
Pulmonary hypertension is a progressive and often fatal disease that frequently presents with dyspnoea on exertion and results in increased right ventricular afterload and right ventricular failure. Although cardiac catheterisation is required for a formal diagnosis, transthoracic echocardiography (TTE) has a central role as a screening tool in those with symptoms and those at risk for developing pulmonary vascular disease. Echocardiographic techniques can be employed to estimate pulmonary artery pressure and resistance, right atrial pressure as well as to derive indirect information about right heart structure and function. Potential causes for pulmonary hypertension may also be identified such as congenital heart disease or left ventricular diastolic dysfunction. An increasing body of evidence has demonstrated the important prognostic utility of echocardiographic data in pulmonary hypertension and highlighted the potential for TTE to help clinicians understand whether treatment responses have been adequate or an escalation in therapy is necessary, as therapeutic options continue to expand for patients with pulmonary arterial hypertension. Although traditional echocardiographic techniques only allow surrogate measures of right ventricular systolic function due to the complex shape of the chamber, newer techniques have enabled three-dimensional assessment of the right ventricle to assess right ventricular volume and contractility. This review will discuss traditional methods as well as newer echocardiographic methods in the setting of pulmonary hypertension.
Topics: Cardiac Catheterization; Echocardiography; Heart Defects, Congenital; Heart Failure; Heart Ventricles; Humans; Hypertension, Pulmonary; Ventricular Function, Right
PubMed: 31109891
DOI: 10.1016/j.hlc.2019.03.003 -
Journal of Cardiac Failure Dec 2021
Topics: Aortic Valve; Aortic Valve Stenosis; Heart Failure; Heart Valve Prosthesis Implantation; Heart Ventricles; Humans
PubMed: 34893203
DOI: 10.1016/j.cardfail.2021.04.005 -
The Journal of Thoracic and... Nov 2022Neonatal interventional strategies for pulmonary atresia with intact ventricular septum are based on tricuspid valve hypoplasia and right ventricle-dependent coronary...
OBJECTIVE
Neonatal interventional strategies for pulmonary atresia with intact ventricular septum are based on tricuspid valve hypoplasia and right ventricle-dependent coronary circulation. We sought to evaluate long-term outcomes comparing biventricular (BiV) versus single-ventricle (SV) strategies.
METHODS
Retrospective review was performed of 119 patients diagnosed with pulmonary atresia with intact ventricular septum from 1995 to 2018. Descriptive statistics summarized patient characteristics and a multivariable Cox survival model was used to compare treatment strategies.
RESULTS
Of 119 patients, 62 (52.1%) were male and 13 (10.9%) had a chromosomal abnormality. BiV was pursued in 53.8% (64 out of 119) and SV in 46.2% (55 out of 119) with median tricuspid valve z scores of -1.59 (interquartile range, -3.03 to 0.21) and -5.12 (interquartile range, -5.60 to -4.06), respectively. The median follow-up was 6 years (interquartile range, 2-15 years). Overall survival at 1, 3, and 10 years was 82.4% (98 out of 119), 80.6% (96 out of 119) and 79.8% (95 out of 119), respectively. End states include 36 (30.3%) BiV, 33 (27.7%) SV, 22 (18.5%) alive without definitive end state, 21 (17.6%) death before end state, 4 (3.4%) 1-and-a-half ventricle, and 3 (2.5%) transplants. No SV were converted to BiV, whereas 4 out of 64 (6.3%) BiV were converted to SV. After adjusting for gender, chromosomal abnormalities, gestational age, and birth weight, SV patients had a significantly higher hazard of mortality (hazard ratio, 9.0; 95% CI, 2.65-30.69; P < .001). Mortality was higher in those with right ventricle-dependent coronary circulation (41.9% [13 out of 31]) compared with those without right ventricle-dependent coronary circulation (7.3% [6 out of 82]) (P < .001).
CONCLUSIONS
Pulmonary atresia with intact ventricular septum remains a challenging lesion for those patients on the SV pathway, particularly with right ventricle-dependent coronary circulation.
Topics: Female; Heart Defects, Congenital; Heart Ventricles; Humans; Infant, Newborn; Male; Pulmonary Atresia; Treatment Outcome; Ventricular Septum
PubMed: 35414413
DOI: 10.1016/j.jtcvs.2021.11.104 -
Circulation. Cardiovascular Imaging Mar 2018Hearts with double outlet right ventricle are a heterogeneous group of malformations in which a comprehensive diagnostic approach is required for tailored surgical... (Review)
Review
Hearts with double outlet right ventricle are a heterogeneous group of malformations in which a comprehensive diagnostic approach is required for tailored surgical management. This pictorial essay revisits essential modifiers of clinical and surgical importance in management of the patients with double outlet right ventricle using 3-dimensional volume-rendered endocardial surface images and 3-dimensional print models. Special emphasis is paid to the infundibular morphology, including the size and orientation of the outlet septum, relative to the margin of the ventricular septal defect, and the extent of the muscular infundibulum as an additional modifier of the distance between the ventricular septal defect margin and the arterial valve or valves.
Topics: Cardiac Surgical Procedures; Double Outlet Right Ventricle; Endocardium; Heart Ventricles; Humans; Imaging, Three-Dimensional; Magnetic Resonance Imaging, Cine; Printing, Three-Dimensional
PubMed: 29855425
DOI: 10.1161/CIRCIMAGING.117.006891 -
BioMed Research International 2018Studies about pulmonary hypertension and congenital heart diseases have introduced the concept of right ventricular remodeling leading these pathologies to a similar... (Review)
Review
Studies about pulmonary hypertension and congenital heart diseases have introduced the concept of right ventricular remodeling leading these pathologies to a similar outcome: right ventricular failure. However right ventricular remodeling is also a physiological process that enables the normal fetal right ventricle to adapt at birth and gain its adult phenotype. The healthy mature right ventricle is exposed to low pulmonary vascular resistances and is compliant. However, in the setting of chronic pressure overload, as in pulmonary hypertension, or volume overload, as in congenital heart diseases, the right ventricle reverts back to a fetal phenotype to sustain its function. Mechanisms include angiogenic changes and concomitant increased metabolic activity to maintain energy production. Eventually, the remodeled right ventricle cannot resist the increased afterload, leading to right ventricular failure. After comparing the fetal and adult healthy right ventricles, we sought to review the main metabolic and cellular changes occurring in the setting of PH and CHD. Their association with RV function and potential impact on clinical practice will also be discussed.
Topics: Chronic Disease; Heart Defects, Congenital; Heart Ventricles; Humans; Hypertension, Pulmonary; Ventricular Dysfunction, Right; Ventricular Remodeling
PubMed: 29581963
DOI: 10.1155/2018/1981568 -
Echocardiography (Mount Kisco, N.Y.) Dec 2017The right ventricle has unique structural and functional characteristics. It is now well recognized that the so-called forgotten ventricle is a key player in... (Review)
Review
The right ventricle has unique structural and functional characteristics. It is now well recognized that the so-called forgotten ventricle is a key player in cardiovascular physiology. Furthermore, there is accumulating evidence that demonstrates right ventricular dysfunction as an important marker of morbidity and mortality in several commonly encountered clinical situations such as heart failure, pulmonary hypertension, pulmonary embolism, right ventricular myocardial infarction, and adult congenital heart disease. In contrast to the left ventricle, echocardiographic assessment of right ventricular function is more challenging as volume estimations are not possible without the use of three-dimensional (3D) echocardiography. Guidelines on chamber quantification provide a standardized approach to assessment of the right ventricle. The technique and limitations of each of the parameters for RV size and function need to be fully understood. In this era of multimodality imaging, echocardiography continues to remain a useful tool for the initial assessment and follow-up of patients with right heart pathology. Several novel approaches such as 3D and strain imaging of the right ventricle have expanded the usefulness of this indispensable modality.
Topics: Echocardiography; Echocardiography, Three-Dimensional; Heart Ventricles; Humans; Ventricular Dysfunction, Right
PubMed: 28833543
DOI: 10.1111/echo.13651 -
International Journal of Cardiology Oct 2016Right ventricular (RV) size, shape and function are distinctly load-dependent and pulmonary load is an important determinant of RV function in patients with congestive... (Review)
Review
Right ventricular (RV) size, shape and function are distinctly load-dependent and pulmonary load is an important determinant of RV function in patients with congestive heart failure (CHF) due to primary impaired left ventricular function and in those with pre-capillary pulmonary hypertension (PH). In a pressure overloaded RV, not only dilation and aggravation of tricuspid regurgitation, but also systolic dysfunction leading to RV failure (RVF) can occur already before the development of irreversible alterations in RV myocardial contractility. This explains RV ability for reverse remodeling and functional improvement in patients with post-capillary and pre-capillary PH of a different etiology, after normalization of loading conditions. There is increasing evidence that RV evaluation by echocardiography in relation with its loading conditions can improve the decision-making process and prognosis assessments in clinical praxis. Recent approaches to evaluate the RV in relation with its actual loading conditions by echo-derived composite variables which either incorporate a certain functional parameter (i.e. tricuspid annulus peak systolic excursion, stroke volume, RV end-systolic volume index, velocity of myocardial shortening) and load, or incorporate measures which reflect the relationship between RV load and RV dilation, also taking the right atrial pressure into account (i.e. "load adaptation index"), appeared particularly suited and therefore also potentially useful for evaluation of RV contractile function. Special attention is focused on the usefulness of RV echo-evaluation in relation to load for proper decision making before ventricular assist-device implantation in patients with CHF and for optimal timing of listing procedures to transplantation in patients with end-stage pre-capillary PH.
Topics: Echocardiography; Heart Failure; Heart Ventricles; Humans; Hypertension, Pulmonary; Prognosis; Ventricular Dysfunction, Right
PubMed: 27474972
DOI: 10.1016/j.ijcard.2016.07.014