Asymptomatic Right Ventricular Hypoplasia in Twin Siblings: A Normal Variant or Cause of Early Mortality?

Qasim, Amna; Dasgupta, Soham; Aly, Ashraf M.

doi:10.1155/2019/6871340

Cited by 3 publications

(2 citation statements)

References 7 publications

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“…Therapeutic management will be established depending on clinical manifestations, associated structural abnormalities, hemodynamic conditions and not the least patient's age. In some author's opinion, if the patient is maintained asymptomatic, no intervention is needed [13].…”

Section:  Surgical Methodsmentioning

confidence: 99%

“…The echocardiography usually shows a RV with three anatomical regions: inlet, trabecular component, and outlet, but with lower volumes and reduced or absent papillary muscles. RV is considered hypoplastic if the length from the tricuspid annulus to the RV apex is less than half the distance from the mitral valve to the LV apex [13].…”

Section: Ultrasound Examinationmentioning

confidence: 99%

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Isolated hypoplastic right ventricle – a challenge in medical practice

Cinteză

Nicolescu²,

Iancu³

et al. 2022

Rom J Morphol Embryol

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Isolated right ventricle hypoplasia (IRVH) is a disease characterized by an underdeveloped right ventricle. It is a congenital heart disease than can associate heterogeneous structural defects and nonspecific clinical features, which can often present a challenging therapeutic management. In this article, there are presented diagnostic methods and treatment options for right ventricle hypoplasia (RVH) according to clinical features, patients age and associated structural heart defects. RVH has a different prognosis in accordance with the severity of the heart defects and the patient's age at which the diagnosis is established. Thus, isolated forms of RVH generally present mild structural and functional defects that can be associated with the onset of symptoms in adolescence or even in adulthood. In these cases, atrial septal defect closure with or without superior cavo-pulmonary anastomosis can be the only procedures needed to correct the hemodynamic abnormalities and relief the symptomatology. Patients with severe form of RVH associated with complex cardiac malformations and onset of the symptoms in the neonatal period require prompt intervention and necessitate palliative procedures. In the long term, these patients could need multiple reinterventions. The family physician should be aware of the cardiac origin of isolated symptoms or clinical signs, such as exertional dyspnea or clubbing fingers, and send the patient for pediatric cardiological evaluation.

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Section:  Surgical Methodsmentioning

confidence: 99%

Section: Ultrasound Examinationmentioning

confidence: 99%

Isolated hypoplastic right ventricle – a challenge in medical practice

Cinteză

Nicolescu²,

Iancu³

et al. 2022

Rom J Morphol Embryol

View full text Add to dashboard Cite

show abstract

Clinical Status and Outcome of Isolated Right Ventricular Hypoplasia: A Systematic Review and Pooled Analysis of Case Reports

et al. 2022

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BackgroundIsolated right ventricular hypoplasia (IRVH), not associated with severe pulmonary or tricuspid valve malformation, is a rare congenital myocardial disease. This study aims to evaluate the clinical status and outcome of IRVH.MethodsA systematic search of keywords on IRVH was conducted. Studies were searched from MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, and Igaku Chuo Zasshi (Ichushi) published between January 1950 and August 2021.ResultsThirty studies met the inclusion criteria. All of these studies were case reports and included 54 patients (25 males and 29 females). The median age of the patients was 2.5 years old (0–15.3 years). Of the 54 patients, 13 (24.1%) reported a family history of cardiomyopathy. Moreover, 50 (92.6%), 19 (35.2%), and 17 (31.5%) patients were diagnosed with cyanosis, finger clubbing, and dyspnea, respectively. Furthermore, 53 (98.2%) patients had a patent foramen ovale or an atrial septal defect (ASD). Z-score of the tricuspid valve diameter on echocardiogram was −2.16 ± 1.53, concomitant with small right ventricular end-diastolic volume. In addition, 29 (53.7%), 21 (38.9%), 7 (13.0%), and 2 (3.7%) patients underwent surgery, ASD closure, Glenn operation, and one and a half ventricular repair, respectively. Among them, nine (20.4%) patients expired, and the multivariable logistic regression analysis showed that infancy, heart failure, and higher right ventricular end-diastolic pressure were risk factors for death.ConclusionsIRVH was diagnosed early in children with cyanosis and was associated with high mortality. This systematic review and pooled analysis provided evidence to assess the of IRVH degree in order to evaluate the clinical status and outcome of IRVH.

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Best management in isolated right ventricular hypoplasia with septal defects in adults

Khajali

Arabian

Aliramezany

2020

J Cardiovasc Thorac Res

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Hypoplastic right ventricle is a rare congenital disease usually associated with pulmonary atresia or tricuspid atresia. Isolated right ventricular hypoplasia is a rare anomaly without important valvular abnormalities. It is associated with inter atrial septal defects leading to the right-to-left shunting of blood. Patients with isolated right ventricular hypoplasia usually have different and variable courses. In some patients, it is recognized in the perinatal period and necessitates prompt intervention; nonetheless, there are some reports of this anomaly in old age with no significant symptoms. In this report, we describe the clinical data and management of 6 adult cases with isolated right ventricular hypoplasia treated medically or surgically based on the severity of the disease and symptoms and then offer an in-depth discussion regarding this rare anomaly.

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Asymptomatic Right Ventricular Hypoplasia in Twin Siblings: A Normal Variant or Cause of Early Mortality?

Cited by 3 publications

References 7 publications

Isolated hypoplastic right ventricle – a challenge in medical practice

Isolated hypoplastic right ventricle – a challenge in medical practice

Clinical Status and Outcome of Isolated Right Ventricular Hypoplasia: A Systematic Review and Pooled Analysis of Case Reports

Best management in isolated right ventricular hypoplasia with septal defects in adults

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