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Pulmonary hypertension among 5 to 18 year old children with sickle cell anaemia in Nigeria.

ABSTRACT:

Background

Pulmonary hypertension (PHT) is a significant cause of mortality in patients with sickle cell disease (SCD). Few studies on PHT in SCD have been carried out in children. This study aimed to estimate the prevalence of PHT in children with sickle cell anaemia (SCA) and determine its clinical and laboratory correlates.

Methods

In this cross sectional study, evaluation involved obtaining bio-data, history and physical examination findings in 175 SCA subjects with haemoglobin genotype SS aged 5 to 18 years and 175 age and sex matched controls with haemoglobin genotype AA. PHT was determined using peak Tricuspid Regurgitant Velocity (TRV) obtained from echocardiography as a marker. Complete blood count (CBC), lactate dehydrogenase (LDH) assay, reticulocyte count, foetal haemoglobin (HbF) estimation as well as Human Immunodeficiency Virus (HIV) I and II, Hepatitis B Virus (HBV) and Hepatitis C Virus (HCV) screening were done for patients with SCA.

Results

The mean peak TRV of subjects with SCA and controls was 2.2 ± 0.4 m/s and 1.9 ± 0.3 m/s respectively and prevalence of PHT among children with SCA and controls was 22.9% and 2.3% respectively. PHT in SCA correlated negatively with body mass index, haematocrit and haemoglobin.

Conclusion

This study affirms that PHT prevalence is high in children with SCA in Nigeria. Cardiovascular examination for signs of PHT is recommended for children with SCA and if required, further echocardiographic assessment from as early as five years.

SUBMITTER: Sokunbi OJ

PROVIDER: S-EPMC5598958 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:BackgroundHemodynamically significant muscular ventricular septal defects in children after the infantile period are a rare occurrence and ideal for transcatheter closure. In cases of severe concomitant pulmonary hypertension, it may be necessary to fenestrate the device. In this report, we present an 18-year old patient with a large mid-trabecular ventricular septal defect and severe pulmonary hypertension that underwent percutaneous closure of the defect with a home-made fenestrated atrial septal occluder.Case presentationAn 18-year-old female patient referred to us with complaints of dyspnea (NYHA score of 2-3). Physical examination revealed an apical rumble and a harsh second heart sound. Echocardiographic examination revealed a large mid-trabecular ventricular septal defect with bidirectional shunt and the widest diameter measuring 22 mm on 2D echocardiography. Left and right heart cavities were enlarged. Before and after the vasoreactivity test performed during cardiac catheterization, average aortic pressure was 65 → 86 mmHg, average pulmonary artery pressure: 58 → 73 mmHg, Qp/Qs: 1.6 → 3.2, PVR: 4.6 → 4.3 Wood/U/m2 and PVR/SVR: 0.5 → 0.2. On left-ventricular angiocardiogram, the largest end-diastolic defect diameter was 21 mm. The closure procedure was performed with transthoracic echocardiographic guidance, using a 24 mm Cera septal occluder and a 14 F sheath dilator to make a 4.5-5 mm opening. Measured immediately after the procedure and during cardiac catheterization one month later, average aortic pressure was 75 → 75 mmHg, average pulmonary artery pressure: 66 → 30 mmHg, Qp/Qs 1.5 → 1.4, PVR: 4.4 → 2.9 Wood/U/m2 and PVR/SVR: 0.4 → 0.2. Transthoracic echocardiographic examination performed 24 hours after the procedure showed a max 35-40 mmHg gradient between the left and right ventricles through the fenestration. After the procedure, we observed sporadic early ventricular systoles and a nodal rhythm disorder that started after approximately 12 hours and spontaneously reverted to normal 9 days later.ConclusionIn patients with large ventricular septal defects, large atrial septal occluders may be used. In cases with risk of pulmonary vascular disease, a safer option would be to close the defect using a manually fenestrated device.

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