Portopulmonary hypertension in children: a rare but potentially lethal and under‐recognized disease

Tingo, Jennifer E.; Rosenzweig, Erika B.; Lobritto, Steven; Krishnan, Usha

doi:10.1177/2045893217723594

Cited by 11 publications

(13 citation statements)

References 27 publications

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“…We speculate that the dominance of CHD-PAH over IPAH in our population might be attributable to the longer survival of patients with PAH, due to cardiac defects, than IPAH patients [24], however, the exact cause of the CHD-PAH dominance is not known. In the present study, we found only one patient with PoPAH (a condition rarely diagnosed in children [25]), and no patient was diagnosed with other forms of associated PAH, including CTD-PAH. Although these types of PAH were also very rare in other registry studies 10 (4.6%) patients with CTD-PAH and three (1.4%) patients with PoPAH in the REVEAL registry [4], our data suggest the need for building an awareness of PAH in pediatricians specializing in children rheumatology and hepatology.…”

Section: Pah Classificationcontrasting

confidence: 50%

Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

Kwiatkowska

Żuk

Migdal

et al. 2020

JCM

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We present the results from the pediatric arm of the Polish Registry of Pulmonary Hypertension. We prospectively enrolled all pulmonary arterial hypertension (PAH) patients, between the ages of 3 months and 18 years, who had been under the care of each PAH center in Poland between 1 March 2018 and 30 September 2018. The mean prevalence of PAH was 11.6 per million, and the estimated incidence rate was 2.4 per million/year, but it was geographically heterogeneous. Among 80 enrolled children (females, n = 40; 50%), 54 (67.5%) had PAH associated with congenital heart disease (CHD-PAH), 25 (31.25%) had idiopathic PAH (IPAH), and 1 (1.25%) had portopulmonary PAH. At the time of enrolment, 31% of the patients had significant impairment of physical capacity (WHO-FC III). The most frequent comorbidities included shortage of growth (n = 20; 25%), mental retardation (n = 32; 40%), hypothyroidism (n = 19; 23.8%) and Down syndrome (n = 24; 30%). The majority of children were treated with PAH-specific medications, but only half of them with double combination therapy, which improved after changing the reimbursement policy. The underrepresentation of PAH classes other than IPAH and CHD-PAH, and the geographically heterogeneous distribution of PAH prevalence, indicate the need for building awareness of PAH among pediatricians, while a frequent coexistence of PAH with other comorbidities calls for a multidisciplinary approach to the management of PAH children.

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Section: Pah Classificationcontrasting

confidence: 50%

Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

Kwiatkowska

Żuk

Migdal

et al. 2020

JCM

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“…Whereas HPS is characterised by increased pulmonary blood flow, low PVR and hypoxaemia, POPH has striking pulmonary vascular remodelling that adversely affects the outcome of orthotopic liver transplantation [23]. POPH appears to be rare in children, with 0–2% of cases reported in PH registries [5, 6, 22, 24].…”

Section: Updates In Paediatric Ph Epidemiology and Classificationmentioning

confidence: 99%

Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management

et al. 2019

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Paediatric pulmonary arterial hypertension (PAH) shares common features of adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for the care of children with PAH, as presented by the Paediatric Task Force of the 6th World Symposium on Pulmonary Hypertension. We provide updates of the current definition, epidemiology, classification, diagnostics and treatment of paediatric PAH, and identify critical knowledge gaps. Several features of paediatric PAH including the prominence of neonatal PAH, especially in pre-term infants with developmental lung diseases, and novel genetic causes of paediatric PAH are highlighted. The use of cardiac catheterisation as a diagnostic modality and haemodynamic definitions of PAH, including acute vasoreactivity, are addressed. Updates are provided on issues related to utility of the previous classification system to reflect paediatric-specific aetiologies and approaches to medical and interventional management of PAH, including the Potts shunt. Although a lack of clinical trial data for the use of PAH-targeted therapy persists, emerging data are improving the identification of appropriate targets for goal-oriented therapy in children. Such data will likely improve future clinical trial design to enhance outcomes in paediatric PAH.

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“…Small paediatric studies suggest a very poor outcome in children. 5 Our aim was to report on our realworld experience of PoPH management in a specialised centre, with the aim of increasing awareness and improving management of these patients.…”

Section: Introductionmentioning

confidence: 99%

Outcome of paediatric portopulmonary hypertension in the modern management era: A case report of 6 patients

Joye¹,

Lador

Aggoun³

et al. 2021

Journal of Hepatology

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Portopulmonary hypertension is a rare but serious complication of portal hypertension or portosystemic shunting. Portopulmonary hypertension is an indication for liver transplantation or shunt closure. However, liver transplantation is contraindicated in patients with severe pulmonary arterial hypertension. Reported mortality rates are high in children with portopulmonary hypertension and there are scarce recommendations on its management. Our aim was to report on our real-world experience of managing portopulmonary hypertension in a specialised centre. We describe a series of 6 children with portopulmonary hypertension. Their median age at diagnosis was 13 years (range 10-15). The underlying liver conditions were cirrhosis of unknown origin (1), congenital portocaval shunts (3), biliary atresia (1), and portal vein cavernoma with surgical mesenterico-caval shunt (1). Median mean pulmonary arterial pressure was 47 mmHg (range 32-70), and median pulmonary vascular resistance was 6.6 Wood units (range 4.3-15.4). All patients except one were treated with a combination of pulmonary arterial hypertension-specific therapy (phosphodiesterase type 5 inhibitors and/or endothelin receptor antagonists and/or prostacyclin analogues). Three patients then benefited from shunt closure and the others underwent liver transplantation. Five patients showed improvement or stabilisation of pulmonary arterial hypertension with no deaths after a mean follow-up of 39 months. Based on our limited experience, early and aggressive treatment with a combination of pulmonary arterial hypertension-specific therapy significantly improves patients' haemodynamic profile and enables the performance of liver transplantation and shunt closure with satisfactory outcomes.

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Portopulmonary hypertension in children: a rare but potentially lethal and under‐recognized disease

Cited by 11 publications

References 27 publications

Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management

Outcome of paediatric portopulmonary hypertension in the modern management era: A case report of 6 patients

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