Platypnea-Orthodeoxia Syndrome Two Decades after Definitive Surgical Repair of Pulmonary Atresia with Intact Ventricular Septum

A 20-year-old female had undergone definitive surgical repair for pulmonary atresia with intact ventricular septum soon after birth. She was referred to our institution with the chief complaint of clubbing fingers. A thorough examination revealed platypnea-orthodeoxia syndrome due to an interatrial right-to-left shunt through a secundum atrial septal defect. Percutaneous closure with an Amplatzer Septal Occluder resulted in resolution of the syndrome.

Síndrome hepatopulmonar como causa de hipoxemia en niños con enfermedad hepática / Hepatopulmonary syndrome as a cause of hypoxemia in hepatic diseases in children

Resumen:Introducción: El síndrome hepatopulmonar es una complicación rara caracterizada por enfermedad hepática asociada con dilataciones vasculares intrapulmonares e hipoxemia. La prevalencia reportada en los escasos estudios realizados en niños con cirrosis es del 3-8%. Aunque es una entidad poco frecuente, es de suma importancia que el clínico la reconozca debido a su curso progresivo.Caso clínico: Se presenta el caso de una niña de 8 años de edad con diagnóstico de cirrosis hepática e hipertensión portal, con sintomatología de disnea y cianosis. Al examen físico se encontró desnutrida, con ictericia generalizada, telangiectasias en abdomen, hipocratismo digital severo, acrocianosis, platipnea y ortodeoxia. La gasometría arterial con PaO2 de 59 mmHg. El ecocardiograma con test de suero salino agitado resultó positivo y la gammagrafía de perfusión pulmonar con macroagregados de albúmina reportó cortocircuito derecha a izquierda del 15%, demostrándose así la existencia de shunt intrapulmonar. Se integró el diagnóstico de síndrome hepatopulmonar de severidad grave, y se recomendó trasplante hepático como el único tratamiento eficaz.Conclusiones: En niños con enfermedad hepática que presenten disnea e hipoxemia, y en los que estén en protocolo de trasplante hepático, debe de buscarse intencionadamente el síndrome hepatopulmonar, ya que el pronóstico dependerá del diagnóstico oportuno.

Abstract:Background: Hepatopulmonary syndrome is a rare complication characterized by liver disease associated with hypoxemia and intrapulmonary vascular dilatations. The prevalence reported in the few studies in children with cirrhosis is 3-8%. Although uncommon, it is important for physicians to recognize this condition because of its progressive course.Case report: We report the case of an 8-year-old girl diagnosed with liver cirrhosis and portal hypertension with symptoms of dyspnea and cyanosis. On physical examination the patient was found malnourished with jaundice, telangiectasias in abdomen, severe clubbing, acrocyanosis, platypnea and orthodeoxia; arterial blood gas showed PaO2 of 59 mmHg. Echocardiography with agitated saline test was positive and lung perfusion scan with albumin macroaggregates reported 15% right-to-left short circuit, thus demonstrating the existence of intrapulmonary shunt. Diagnosis of severe hepatopulmonary syndrome was made. Liver transplantation is recommended as the only effective treatment.Conclusions: In children with liver disease presenting dyspnea and hypoxemia and those enrolled in a liver transplant protocol, hepatopulmonary syndrome must be intentionally searched because the prognosis will depend on timely diagnosis.

Platypnea-orthodeoxia syndrome in a patient with a pre-existing patent foramen ovale successfully treated with an atrial septal occluder / 老年心脏病学杂志（英文版）

Platypnea orthodeoxia syndrome is associated with dyspnea and arterial oxygen desaturation accentuated by an upright posture. It can be secondary to an intracardiac shunt. We report a case of platypnea-orthodeoxia syndrome (POS) in a 58-year old male patient who had a pre-existing patent foramen ovale (PFO) and substantial pulmonary pathologies. He was successfully treated by percutaneous transcatheter closure of the PFO. Our case highlights the importance of recognition of this rare syndrome in patients who present with unexplained hy-poxia for whom transcatheter closure of the interatrial shunt can be safely carried out.

Transcatheter Treatment of Atrial Septal Defect Presenting with Platypnea-Orthodeoxia Syndrome

A 29-year-old woman was referred to our institute for symptomatic hypoxemia. Her dyspnea was aggravated while sitting or standing and relieved while in supine position. She did not have any pulmonary disease. Transthoracic echocardiography and heart computed tomography revealed an underestimated small atrial septal defect (ASD) with a left-to-right shunt. A cardiac catheterization was performed to evaluate pulmonary hypertension. It revealed a normal pulmonary artery pressure and a large ASD with bidirectional shunt during Valsalva maneuver by intracardiac echocardiogram. Her arterial oxygen saturation decreased from 93% while supine to 79% while at a 15degrees sitting position. Thus, the patient was diagnosed with platypnea-orthodeoxia syndrome. The ASD was successfully closed with Amplatzer(R) (St. Jude Medical) septal occluder and both platypnea and orthodeoxia were resolved immediately after the procedure.

Síndrome de ortodeoxia-platipnea y embolia luego de endocarditis derecha complicada: consecuencias del Foramen Oval Permeable / Platypnea-Orthodeoxia syndrome and embolism after complicated right side endocarditis

Se presenta el caso clínico de una mujer de 63 años de edad con Endocarditis de la Válvula Tricúspide que desarrolló tromboembolismo pulmonar y un síndrome de platipnea-ortodeoxia explicado por la presencia de un formen oval permeable. La paciente se recuperó satisfactoriamente después de un reemplazo de la válvula tricúspide.

The case of 63 year old woman with Endocarditis at the Tricuspid Valve, pulmonary and paradoxical embolism with platypnea-orthodeoxia syndrome is presented. The patient recovered after surgical replacement of the tricuspid valve.

A Case of Platypnea-Orthodeoxia Syndrome Caused by a Patent Foramen Ovale / 日本心臓血管外科学会雑誌

We encountered a patient with platypnea-orthodeoxia syndrome. This rare syndrome is characterized by right-to-left shunt, which appears in the upright position. A 76-year-old woman with symptomatic hypoxemia was referred for evaluation and treatment. She did not complain of dyspnea while in a supine position, but experienced dyspnea with severe hypoxemia in a sitting or standing position. She did not have any pulmonary diseases that can be cause of dyspnea. Echocardiography revealed a patent foramen ovale and mild left-to-right shunt when the patient was supine. However in an upright position, right-to-left shunt appeared and the arterial oxygen saturation dropped from 95% to 80% with dyspnea. Cardiac catheterization revealed normal pulmonary artery pressure and right-to-left shunt through the patent foramen ovale in the sitting position. We then diagnosed platypnea-orthodeoxia syndrome. The chest CT showed deformity of the right atrium caused by compression of the elongated ascending aorta. The patent foramen ovale was closed and the ascending aorta was shortened by open heart surgery. Her dyspnea and hypoxemia in the upright position was completely resolved after surgery.

A Case of Atrial Septal Aneurysm Associated with Multiple Renal Infarction

Atrial septal aneurysm (ASA) was reported as a cause of unknown origin of central or peripheral thromboembolism with patent foramen ovale, aortic debris and spontaneous echo contrast. Especially the paradoxical right to left shunt via micro-fenestration on this sac may be the important cause of this embolism. We report a case of 39 year-old female patient who had atrial septal aneurysm with multiple renal infarction. Symptom was improved with aneurysmectomy and patch closure, then long term anticoagulation has been continued.