Isolated hypoplastic right ventricle – a challenge in medical practice

Abstract

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.

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

“…Treatment options range from medical therapy to Fontan surgery, and in this case, the recorded RV dimensions were the main criteria for choosing the surgical method [4,5]. The majority of patients recorded in cases in the literature underwent ASD closure, but those patients had arterial oxygen saturation compared to patients who underwent Glenn operation or one-and-a-half ventricular repair [2].…”

“…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].…”

“…The echocardiography examination revealed a closed DA and a right-left shunt on the ASD. 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].…”

“…An associated atrial septal defect (ASD) or a patent foramen ovale (PFO) could persist and represent an escape valve, resulting in cyanosis. The clinical presentation depends on the degree of RV hypoplasia, interatrial communication size, and persistent pulmonary hypertension, and it has a broad outcome spectrum, from death in early infancy to mild cyanosis [2][3][4].…”

“…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].…”