Non Invasive Assessment of Pulmonary Artery Pressure in Children with Portal Hypertension
Authors: El-din El Shazly Lerine Bahy , Talaat Iman Mohamed , Abd Elhady Nasef Marwa Waheed , Ali Elhanfy Lamiaa Ali Mohammed 
Published: July 2024

Abstract

Background
Pulmonary artery hypertension is a complication of liver cirrhosis with Portal Hypertension (PHT). It has been mentioned in case reports or small series of children. Fewinformation known on its prevalence and long-term survival in childhood liver disease. This study will assess the Pulmonary Atery Pressure (PAP) in children with PHT in Pediatric Hepatologyclinic, Ain Shams University Children Hospital in order to identify clinical characteristics of Porto PH and its prevalence among those children.

Aim of the Work
To assess the PAP using Echocardiography in children with PHT who attend Pediatric Hepatology clinic, Ain Shams University Children Hospital.

Patients and Methods
This case-control study was conducted on thirty patients enrolled from Pediatric Hepatology clinic, Ain Shams University Children Hospital in a period of one year from June 2021 to June 2022 according to inclusion criteria : history of hematemesis and melena, clinical examination: Splenomegaly, dilated anterior abdominal wall vessels, ascites ± firm hepatomegaly and imaging: Ultrasonography (US) /color Doppler-US (CDUS): Splenomegaly, presence of Porto collateral vessels and ascites. This information will be completed with data on liver size, echo texture, and margins which can suggest underlying cirrhosis, portal vein diameter, portal blood flow velocity, direction of blood flow in portal vein with age ranging from 1 to 16 years with median (IQR) of 8 (4.5-12) years; they were 11 females (36.7%) and 19 males (63.3%) and thirty healthy matched age and sex as a control group.

Results
In our study, MPAP was 9.79 ± 2.21 mmHg (7.2-18.2 mmHg). Statistically, there was statistically significant increase in the level of TR velocity, TR PG, RVSP and MPAP in patients with PHT than control group with p-value &lt;0.001, 0.001; 0.001 and &lt;0.001; respectively. Also, there was statistically significant increase in the percentage of patients with abnormal TR PG and patients with elevated systolic PAP in patients with PHT (20.0%) than control group (0.0%) with p-value = 0.010 and 0.010; respectively. Also, there was statistically significant decrease in the level of PA acceleration time (ms) in patients with PHT than control group with p-value &lt;0.001. Also, there was statistically significant increase in the level of PAEDP (mmHg) and LA (cm) in patients with PHT than control group with p-value &lt;0.001 and 0.014; respectively. In our studied group of patients we did not found any of them has PH despite the higher parameters of their Echo studies and that needs follow up of those patients. So the prevalence of PH in those patients is 0%.

Conclusion
PHT is an important risk factor of development of PH in children. Transthoracic echocardiography can be reliably used as an initial non-invasive, safe and reliable modality for the assessment of pulmonary artery hypertension in children, and can obviate the need of right heart catheterization in some patients, especially those with mild PHT.