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

Khajali, Zahra; Arabian, Maedeh; Aliramezany, Maryam

doi:10.34172/jcvtr.2020.36

Cited by 6 publications

(7 citation statements)

References 22 publications

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“…Cases of mild hypoplastic RV disease can be associated with late onset of the cyanosis and can be surgically corrected by ASD closure. Meanwhile, severe RVH associated with valvular abnormalities is characterized by early onset of cyanosis [10]. In two of our cases, considered mild forms, they presented with cyanosis at exertion and dyspnea.…”

Section:  Clinical Presentationmentioning

confidence: 60%

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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:  Clinical Presentationmentioning

confidence: 60%

“…For patients with hypoplastic RV that associate severe structural abnormalities, total cavo-pulmonary anastomosis, Fontan procedure is the only solution [10]. The Fontan procedure is the final palliative surgical method for patients with congenital cardiac malformations which associate unique anatomical or functional ventricle.…”

Section:  Surgical Methodsmentioning

confidence: 99%

Isolated hypoplastic right ventricle – a challenge in medical practice

Cinteză

Nicolescu²,

Iancu³

et al. 2022

Rom J Morphol Embryol

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“…Today, due to advances in surgery and treatment methods, it is expected that the survival rate of infants with congenital heart disease will increase and the population of adults with this problem is increasing (6).…”

Section: Discussionmentioning

confidence: 99%

Case report of unusual presentation of COVID-19 in a patient with pulmonary hypertension

Moazenzadeh

Aliramezany

2022

J Emerg Pract Trauma

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Objective: COVID-19 has now become a worldwide health problem with many new cases diagnosed every day. people with underlying heart diseases are more likely to get infected and have poor prognosis. Of them, adult patients with congenital heart disease need special attention due to their different symptoms and course of the disease. Case Presentation: Here, we report the unusual presentation and course of COVID-19 disease with rapid progression of right ventricle failure and pulmonary hypertension in patient who was infected with new SARS-CoV-2 virus. She is a 39-year-old woman, known case of ventricular septal defect and pulmonary hypertension with a history of open-heart surgery at the age of seven that arrived to our clinic due to exacerbation of dyspnea; progressive lower extremity edema and mild ascites from 10-days ago. Conclusion: Any changes in the condition of adult patients with congenital heart disease in this epidemic should be considered as a potential infection by this virus and the necessary and accurate evaluation should be done.

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“…She became hypotensive and oliguric, so dopamine (5 mcg/kg/min) was added to her therapy. highlight the necessity of surgical treatment-ASD closure, systemic-pulmonary shunt (SPS), Glenn anastomosis, one-and-a-half repair, and Fontan circulation [2][3][4][5][6].…”

Section: Case Reportmentioning

confidence: 99%

“…The natural history of this abnormality Is mostly relatively benign, with spontaneous RV and pulmonary circulation recovery. On the other hand, the data in the literature 2 of 9 highlight the necessity of surgical treatment-ASD closure, systemic-pulmonary shunt (SPS), Glenn anastomosis, one-and-a-half repair, and Fontan circulation [2][3][4][5][6].…”

Section: Introductionmentioning

confidence: 99%

Percutaneous Modified Blalock–Taussig Shunt Closure in a Patient with Isolated Right Ventricular Hypoplasia

Krasic,

Dizdarevic,

Vranic

et al. 2023

JCDD

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Clinical presentation, course, and treatment for patients with isolated right ventricular (RV) hypoplasia (IRVH) depends on the degree of hypoplasia that is present—this is a spectrum from spontaneous maturation to Fontan circulation over time. An 8-month-old infant presented with IRVH; in the patient, a modified Blalock–Taussig (MBTS) shunt was closed percutaneously after spontaneous RV function recovery. A female newborn was diagnosed with differential cyanosis at birth. The echocardiography showed a hypertrophic RV with a small cavity, a right–left shunt on the atrial septal defect, an almost closed ductus arteriosus (DA), and a small tricuspid valve ring (Z-score-2) with mild regurgitation (pressure gradient 30 mmHg). On the 4th day of life, the patient showed deepened cyanosis and hyperlactatemia was registered. The echocardiography examination revealed a closed DA. Right ventriculography performed on the 5th day of life evidenced the presence of a small hypertrabeculated RV. The pressure in the RV increased. A right-side MBTS was created on the 6th day of life. Further echocardiographic findings indicated a gradual development of the RV and a decrease in RV pressure. MBTS occlusion was performed when the patient was 8 months old. Vital parameters were monitored invasively and noninvasively after the balloon occlusion of MBTS. Percutaneous MBTS occlusion was successfully performed using an Amplatzer vascular plug 2 (AVP2). During the follow-up period, the patient was found to have maintained a normal percutaneous oxyhaemoglobin blood saturation.

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

Cited by 6 publications

References 22 publications

Isolated hypoplastic right ventricle – a challenge in medical practice

Isolated hypoplastic right ventricle – a challenge in medical practice

Case report of unusual presentation of COVID-19 in a patient with pulmonary hypertension

Percutaneous Modified Blalock–Taussig Shunt Closure in a Patient with Isolated Right Ventricular Hypoplasia

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