Clinical, hemodynamic, echocardiographic, angiographic profiles and post-operative outcomes among DCRV patients from a tertiary care referral center in India

Background: This retrospective study of data from 2006 to 2018 at a tertiary care referral center in India aims to document the contemporary clinical and hemodynamic profile of patients who were diagnosed with double chamber right ventricle (DCRV) based on echocardiography and cardiac catheterization. Patients were followed up and their outcomes were studied to document their short and long term outcomes.Methods: It is a retrospective observational study of patients diagnosed with DCRV in a tertiary care hospital in India. The diagnosis of DCRV was based on the following criteria: an echocardiographic diagnosis of DCRV with doppler evidence of a mid ventricular gradient; cardiac catheterization revealing a systolic pressure gradient between right ventricular inflow and outflow tracts; a right ventricular angiogram demonstrating an anomalous muscle bundle causing obstruction well below the infundibulum. All the patients were followed up for their long term outcomes.Results: All the patients underwent echocardiography and cardiac catheterization for confirmation of the diagnosis. Sixty percent of our patients presented during adulthood, which is very unusual presentation of this disease entity. Median age of our patient cohort was 23.5 years. Patients presenting during adulthood have atypical symptoms. Dyspnea was the most common presenting symptom in this study. Right ventricular hypertrophy (73.3%)and right bundle branch pattern (26.6%) were the common electrocardiographic findings in our patients. DCRV is commonly associated with other anomalies. Ventricular septal defect (VSD) was the commonest associated anomaly, which was seen in 80% of our patients. Mean gradient cross the anomalous muscle bundle was 67.5 mmHg. Three of our patients (20%) had no associated anomaly, which is very rare in DCRV. Eight patients underwent surgical correction with significant reduction in gradients in all and no perioperative mortality. Median follow up of 8 years showed no adverse outcomes and no progression of gradients.Conclusions: This study describes in detail the clinical profile, echocardiographic and angiographic identification of anomalous muscle bundles in DCRV patients, which will help the young readers in identifying this often missed diagnosis. It highlights the unusual presentation during adulthood with atypical symptoms in DCRV patients with excellent long-term outcomes on follow up.

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Tu1194 Liquid Nitrogen Spray Cryotherapy (SCT) Based Multimodal Endoscopic Management of Dysplastic Barrett’s and Foregut Neoplasia: Retrospective Review and Long Term Follow-up at a Single Tertiary Care Referral Center

Gastrointestinal Endoscopy ◽

10.1016/j.gie.2016.03.1173 ◽

2016 ◽

Vol 83 (5) ◽

pp. AB570

Author(s):

Shivangi Kothari ◽

Krystle Bittner ◽

Asad Ullah ◽

Vivek Kaul

Keyword(s):

Liquid Nitrogen ◽

Retrospective Review ◽

Tertiary Care ◽

Endoscopic Management ◽

Referral Center ◽

Long Term Follow Up

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Right Ventricular Anomalous Muscle Bundle

Circulation ◽

10.1161/01.cir.43.1.115 ◽

1971 ◽

Vol 43 (1) ◽

pp. 115-127 ◽

Cited By ~ 53

Author(s):

J. W. FORSTER ◽

J. O. HUMPHRIES

Keyword(s):

Right Ventricular ◽

Muscle Bundle ◽

Anomalous Muscle ◽

Anomalous Muscle Bundle

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Right Ventricular Anomalous Muscle Bundle

Circulation ◽

10.1161/01.cir.44.5.969 ◽

1971 ◽

Vol 44 (5) ◽

pp. 969-969 ◽

Cited By ~ 1

Author(s):

G. E. GALE ◽

J. B. BARLOW

Keyword(s):

Right Ventricular ◽

Muscle Bundle ◽

Anomalous Muscle ◽

Anomalous Muscle Bundle

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245 Long-term follow-up of pacing and sensing parameters in right ventricular dysplasia patients with implantable cardioverter defibrillator

EP Europace ◽

10.1016/s1099-5129(05)80154-0 ◽

2005 ◽

Vol 7 ◽

pp. 52-52

Keyword(s):

Right Ventricular ◽

Implantable Cardioverter Defibrillator ◽

Cardioverter Defibrillator ◽

Right Ventricular Dysplasia ◽

Long Term Follow Up

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Mid- to long-term follow-up of pulmonary valve replacement with BioIntegral injectable valve

European Journal of Cardio-Thoracic Surgery ◽

10.1093/ejcts/ezaa337 ◽

2020 ◽

Author(s):

Simone Ghiselli ◽

Cristina Carro ◽

Nicola Uricchio ◽

Giuseppe Annoni ◽

Stefano M Marianeschi

Keyword(s):

Cardiopulmonary Bypass ◽

Right Ventricular ◽

Tetralogy Of Fallot ◽

Valve Replacement ◽

Pulmonary Valve ◽

Pulmonary Stenosis ◽

Pulmonary Regurgitation ◽

Pulmonary Valve Replacement

Abstract OBJECTIVES Chronic pulmonary valve (PV) regurgitation is a common late sequela after repair of congenital heart diseases like tetralogy of Fallot or pulmonary stenosis, leading to right ventricular dilatation and failure and increased late morbidity and mortality. Timely reoperation may lead to a complete right ventricular recovery. An injectable PV allows pulmonary valve replacement, with or without cardiopulmonary bypass, under direct observation, thereby minimizing the impact of surgery on cardiac function. The aim of this study was to evaluate the feasibility and mid- to long-term clinical outcomes with this device. METHODS From April 2007 to October 2019, a total of 85 symptomatic patients with severe pulmonary regurgitation or pulmonary stenosis underwent pulmonary valve replacement with an injectable stented pulmonary prosthesis. Data were collected from the international proctoring registry. Mean patient age was 26.7 years. The underlying diagnosis was repaired tetralogy of Fallot in 69.4% patients; moderate or severe pulmonary regurgitation was present in 72.9%. All patients had echocardiographic scans before the operation and during the follow-up period. A total of 54.1% patients also had preoperative/postoperative cardiac magnetic resonance imaging (MRI) or catheterization; 25.9% had off-pump implants. In 53% patients, pulmonary valve replacement was associated with the repair of other cardiac defects. RESULTS Minor postoperative complications were observed in 10.8% patients. The overall mortality rate was 2.3%; mortality after valve replacement was linked to a severe cardiac insufficiency and it was not related to a prosthesis failure; 1 prosthesis was explanted from 1 patient because of endocarditis, and 6% of patients developed PV stenosis; minor complications occurred in 4.8%. The mean follow-up period was 4.8 years (2 months–12.7 years); 42% of the patients were followed for more than 5 years. Follow-up echocardiography and cardiac MRI showed a significant reduction in RV size and low gradients across the PV. CONCLUSIONS An injectable PV may be implanted without cardiopulmonary bypass and in a hybrid operating theatre with minimal surgical impact. The bioprosthesis, available up to large sizes, has a low profile, laminar flow and no risk of coronary artery compression. Incidence of endocarditis is rare. The lack of a suture ring permits the implant of a relatively larger prosthesis, thereby avoiding a right ventricular outflow tract obstruction. This device permits future percutaneous valve-in-valve procedures, if needed. Results concerning durability are encouraging, and mid- to long-term haemodynamic performance is excellent.

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INDUCTION OF LV DYSSYNCHRONY BY RIGHT VENTRICULAR APICAL PACING IS ASSOCIATED WITH INCREASED ALL-CAUSE MORTALITY AND HEART FAILURE HOSPITALIZATIONS AT LONG-TERM FOLLOW-UP

Journal of the American College of Cardiology ◽

10.1016/s0735-1097(12)60641-x ◽

2012 ◽

Vol 59 (13) ◽

pp. E640

Author(s):

Ulas Höke ◽

Dominique Auger ◽

Darryl Leong ◽

Nina Marsan ◽

E.R. Holman ◽

...

Keyword(s):

Heart Failure ◽

Right Ventricular ◽

Lv Dyssynchrony ◽

All Cause Mortality ◽

Long Term Follow Up ◽

Right Ventricular Apical Pacing

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Nephrocalcinosis, Renal Dysfunction, and Calculi in Patients With Primary Hypoparathyroidism on Long-Term Conventional Therapy

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/clinem/dgz319 ◽

2020 ◽

Vol 105 (4) ◽

pp. e1215-e1224 ◽

Cited By ~ 2

Author(s):

Soma Saha ◽

Devasenathipathy Kandasamy ◽

Raju Sharma ◽

Chandrasekhar Bal ◽

Vishnubhatla Sreenivas ◽

...

Keyword(s):

Renal Dysfunction ◽

Conventional Therapy ◽

Care Center ◽

Tertiary Care ◽

Fractional Excretion ◽

Renal Calculi ◽

Serum Total Calcium ◽

Calcium Phosphorus

Abstract Context There are concerns about the long-term safety of conventional therapy on renal health in patients with hypoparathyroidism. Careful audit of these would help comparisons with upcoming parathyroid hormone therapy. Objective We investigated nephrocalcinosis, renal dysfunction, and calculi, their predictors and progression over long-term follow-up in patients with primary hypoparathyroidism (PH). Design and Setting An observational study at a tertiary care center was conducted. Participants and Methods A total of 165 PH patients receiving conventional therapy were evaluated by radiographs, ultrasonography, and computed tomography. Their glomerular filtration rate (GFR) was measured by Tc-99m-diethylenetriamine penta-acetic acid clearance. Clinical characteristics, serum total calcium, phosphorus, creatinine, hypercalciuria, and fractional excretion of phosphorus (FEPh) at presentation and during follow-up were analyzed as possible predictors of renal complications. Controls were 165 apparently healthy individuals. Results Nephrocalcinosis was present in 6.7% of PH patients but not in controls. Patients younger than 15 years at presentation and with higher serum calcium-phosphorus product were at higher risk. Nephrocalcinosis showed no significant association with cataract and intracranial calcification. Prevalence of renal calculi was comparable between hypoparathyroid patients and controls (5% vs 3.6%, P = .58). Fourteen percent of patients had a GFR less than 60 mL/min/1.73 m2. Increased FEPh during follow-up was the significant predictor of low GFR. Nephrocalcinosis developed in 9% of patients over 10 years of conventional therapy. Conclusion A total of 6.7% of PH patients had nephrocalcinosis, and 14% showed renal dysfunction. Prevalence of renal calculi was similar in patients and controls. Nine percent of patients developed nephrocalcinosis over 10 years of conventional therapy.

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