Pulmonary Hypertension that Developed During Treatment for Hepatopulmonary Syndrome and Pulmonary Arteriovenous Malformation

Ikubo, Yumiko; Kasai, Hajime; Sugiura, Toshihiko; Saito, Tatsuhito; Shoji, Hiroki; Sakao, Seiichiro; Kasahara, Yasunori; Tanabe, Nobuhiro

doi:10.2169/internalmedicine.1993-18

Cited by 6 publications

(3 citation statements)

References 12 publications

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“…This patient did not have prior HPS and post-mortem autopsy revealed intimal and medial hyperplasia of the pulmonary vasculature without evidence of pulmonary emboli. Since this publication, there have been approximately 14 documented cases in the literature of this phenomenon, including five pediatric patients [ 4 , 7 - 15 ]. Authors have postulated two main mechanisms by which this phenomenon occurs.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Hypertension as a Rare Complication After Orthotopic Liver Transplant in a Patient With Non-alcoholic Steatohepatitis (NASH) Cirrhosis Complicated by Hepatopulmonary Syndrome

Dadlani¹,

Eiswerth²,

Bosch³

et al. 2022

Cureus

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Hepatopulmonary syndrome is a phenomenon that results in an intrapulmonary shunt leading to dyspnea and hypoxemia with poor response to oxygen supplementation. It is now an indication for liver transplantation; however, some transplants result in subsequent pulmonary hypertension. Postulated mechanisms include unmasking of underlying pulmonary hypertension with liver transplant and increased pulmonary vascular resistance due to increased blood flow in hepatopulmonary syndrome. In this case, we describe pulmonary hypertension developing after orthotopic liver transplant in a cirrhotic patient with hepatopulmonary syndrome.

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Section: Discussionmentioning

confidence: 99%

Pulmonary Hypertension as a Rare Complication After Orthotopic Liver Transplant in a Patient With Non-alcoholic Steatohepatitis (NASH) Cirrhosis Complicated by Hepatopulmonary Syndrome

Dadlani¹,

Eiswerth²,

Bosch³

et al. 2022

Cureus

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“…The main clinical manifestations are dyspnea and cyanosis. At this point, the exact pathogenesis of HPS is still unclear, and is most likely thought to occur due to vasodilatation caused by nitric oxide[ 19 ], which increases pulmonary blood flow and cardiac output, leading to pulmonary ventilation-perfusion mismatch and arteriovenous shallows, ultimately making it difficult for oxygen molecules to bind with hemoglobin, and resulting in hypoxemia[ 20 , 21 ]. Currently, liver transplantation is the accepted treatment.…”

Section: Discussionmentioning

confidence: 99%

Growth hormone ameliorates hepatopulmonary syndrome and nonalcoholic steatohepatitis secondary to hypopituitarism in a child: A case report

Zhang¹,

Yuan²,

Fang³

et al. 2022

WJCC

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BACKGROUND Craniopharyngioma is a benign tumor that usually develops in children; however, it is located in the center and close to sensitive structures, such as the pituitary gland and hypothalamus. As the hypothalamus plays a crucial role in the homeostasis of anterior pituitary hormone synthesis, damage to the hypothalamus leads to multiple pituitary hormone deficiencies and non-alcoholic fatty liver disease, including hepatopulmonary syndrome (HPS). HPS has limited treatment and poor prognosis. CASE SUMMARY A girl aged 13 years and 6 mo underwent surgery for craniopharyngioma 6 years prior. Right craniotomy was performed with total resection via the corpus callosum approach, and the tumor at the base was approximately 3.5 cm × 3.5 cm × 4.0 cm. At 1 year postoperatively, she exhibited abdominal distension and weakness, and the laboratory tests revealed fatty liver disease. Thereafter, she had not visited the outpatient clinic for 2 years. Two years ago, she developed decreased activity endurance, severe cyanosis, chest tightness, wheezing, and intermittent and recurrent low fever after mild physical labor. Hepatobiliary ultrasonography, liver biopsy, and contrast echocardiography of the right heart showed cirrhosis and multiple pituitary hormone deficiencies, indicating HPS. After 1 year of treatment with recombinant human growth hormone, the liver function and oxygenation improved; she did not undergo liver transplantation. CONCLUSION Craniopharyngioma surgery can easily cause hypopituitarism, which can lead to nonalcoholic steatohepatitis and HPS in children. Early growth hormone therapy is important to improve the prognosis of these diseases.

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“…PAVMs, particularly multiple and/or diffuse PAVMs, can be part of a syndrome, often familial, such as hereditary hemorrhagic telangiectasia (HHT) [ 1 , 2 ]. Acquired PAVMs have been reported in hepatopulmonary syndrome (HPS) and patent ductus venosus (PDV) [ 3 – 5 ]. PAVMs are most frequently asymptomatic, but may be associated with hemoptysis, dyspnea, and hypoxemia due to paradoxical embolization.…”

Section: Introductionmentioning

confidence: 99%

Effective long-term sirolimus treatment in hypoxemia mainly due to intrapulmonary right-to-left shunt in a patient with multiple vascular anomalies

Liu

Duan

Yin

et al. 2023

Orphanet J Rare Dis

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Pulmonary arteriovenous malformations (PAVMs), particularly where feeding artery/arteries to PAVMs ≥ 3 mm can be treated with embolization. The treatment for hypoxemia resulting from multiple small or diffuse PAVMs remains unclear.We report a girl aged 5 years and 10 months presented with cyanosis and decreased activity after exercise (83–85% of pulse oxygen saturation, SpO2). She had 1 skin lesion on her face and 1 suspected hemangioma on her left upper extremity at birth and that gradually disappeared spontaneously. Physical examination revealed clubbed fingers, and abundant vascular networks on her back. Contrast-enhanced lung CT (slice thickness:1.25 mm) with vascular three-dimensional reconstruction and abdominal CT revealed increased bronchovascular bundles, increased diameter of the pulmonary artery and ascending aorta, and intrahepatic portosystemic venous shunts due to patent ductus venosus. Echocardiography revealed increased diameter of aortic and pulmonary artery. Transthoracic contrast echocardiography was highly positive (bubble appearing in the left ventricle after 5 cardiac cycles). Abdominal doppler ultrasound revealed hepatic-portal venous shunt. Magnetic resonance imaging, artery and vein of the brain revealed multiple malformations of venous sinuses. The patient received sirolimus for 2 years and 4 months. Her condition improved significantly. SpO2 gradually increased to 98%. Her finger clubbing gradually normalized.Our report implicates sirolimus might be a potential treatment option in persistent hypoxemia mainly due to intrapulmonary right-to-left shunt even small multiple or diffusive PAVMs in pediatric patients with multiple cutaneous and visceral vascular anomalies.

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Pulmonary Hypertension that Developed During Treatment for Hepatopulmonary Syndrome and Pulmonary Arteriovenous Malformation

Cited by 6 publications

References 12 publications

Pulmonary Hypertension as a Rare Complication After Orthotopic Liver Transplant in a Patient With Non-alcoholic Steatohepatitis (NASH) Cirrhosis Complicated by Hepatopulmonary Syndrome

Pulmonary Hypertension as a Rare Complication After Orthotopic Liver Transplant in a Patient With Non-alcoholic Steatohepatitis (NASH) Cirrhosis Complicated by Hepatopulmonary Syndrome

Growth hormone ameliorates hepatopulmonary syndrome and nonalcoholic steatohepatitis secondary to hypopituitarism in a child: A case report

Effective long-term sirolimus treatment in hypoxemia mainly due to intrapulmonary right-to-left shunt in a patient with multiple vascular anomalies

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