Este artigo descreve um paciente que se apresentou com quadro de hipoxemia e platipnéia e cujo único achado na investigação foi a presença de um forame oval patente com shunt direita-esquerda sem hipertensão pulmonar, caracterizando uma síndrome rara conhecida como platipnéia-ortodeoxia, de interessantes características fisiopatológicas e com opções terapêuticas ainda não totalmente definidas.
Shunt
Hospital de Clínicas de Porto Alegre - Porto Alegre, RS
Este artigo descreve um paciente que se apresentou com quadro de hipoxemia e platipnéia e cujo único achado na investigação foi a presença de um forame oval patente com
Casos de hipoxemia secundária a
Paciente do sexo masculino, 71 anos, branco, casado, atendido na Emergência do Hospital de Clínicas de Porto Alegre (HCPA) por dispnéia importante em repouso, sem sintomas associados. Referia dispnéia progressiva há 10 meses, com aumento de intensidade há 10 dias.
Na história médica pregressa referia: tabagismo dos 31 aos 35 anos; contusão torácica sem seqüelas há 25 anos; hipertensão arterial sistêmica desde 1985 em tratamento com amlodipina, hidroclortiazida e enalapril; dislipidemia desde 1996 em uso de sinvastatina e doença do refluxo gastroesofágico em uso de omeprazol.
Ao exame físico, apresentava-se consciente, orientado, taquipnéico (FR = 28 irm), com Sat.O2 = 88% em ar ambiente, sentado. Apresentava PA = 140/80 mmHg, FC = 96 bpm, com ritmo cardíaco regular, dois tempos, bulhas normofonéticas, sem sopros. Os pulsos periféricos dos quatro membros eram palpáveis e simétricos, com extremidades aquecidas e com cianose. Não apresentava outras alterações.
Os exames mostravam: gasometria arterial, em ar ambiente, com pH = 7,53; pCO2 = 24,9; HCO3 = 20,3; PO2 = 51,7; Sat.O2 = 90,5%; hematócrito = 50%, hemoglobina = 16,5 g/dl; leucograma e plaquetas normais; enzimas cardíacas normais; ECG sem alterações isquêmicas; RX tórax com calcificações pleurais e angiotomografia de tórax sem evidências de tromboembolia pulmonar.
Evoluiu com piora progressiva da hipoxemia, necessitando internação no Centro de Terapia Intensiva (CTI), ventilação mecânica não-invasiva, e posteriormente invasiva, com duas tentativas sem sucesso de extubação.
Prosseguindo a investigação, foram realizadas: cintilografia pulmonar perfusional, com baixa probabilidade para tromboembolia pulmonar; Doppler de membros inferiores sem evidências de trombose venosa profunda; tomografia de tórax de alta resolução sem alterações que justificassem o grau de hipoxemia; ecocardiograma transtorácico com contraste aerado, mostrando aneurisma de septo interatrial, com passagem intensa e precoce de contraste para cavidades esquerdas, compatível com forame oval patente, sem evidências de hipertensão arterial pulmonar, ventrículo esquerdo (VE) hipertrófico, com déficit de relaxamento, função sistólica global e segmentar preservadas e com fração de ejeção (FE) = 66%; ecocardiograma transesofágico, com Doppler, com evidências de forame oval permeável com fluxo predominante direita-esquerda, sem trombos intracavitários; arteriografia pulmonar com ausência de
Com a hipótese diagnóstica de hipoxemia secundária ao
Como persistisse apresentando dessaturação quando colocado em posição sentada, e, com isso, necessitando ainda de ventilação mecânica a despeito da ausência de um gradiente de pressão direita esquerda, o paciente foi submetido a correção cirúrgica do forame oval patente (atriosseptoplastia) (
Esse relato descreve um homem de 71 anos com quadro clínico de platipnéia-ortodeoxia causado por
A causa de
A síndrome clínica descrita como platipnéia-ortodeoxia constitui-se em um quadro infreqüente, mas também pouco valorizado, e apresenta-se com dispnéia e hipoxemia associados à posição supina e sentada e melhora sintomática quando o paciente assume uma postura deitada. A primeira descrição desse quadro foi feita por Burchell em 1949 e desde então menos de uma centena de casos foi descrita4.
Sob condições normais, uma comunicação interatrial permite fluxo esquerda-direita, movido pelo gradiente pressórico e por uma maior complacência das câmaras direitas em comparação com as esquerdas. Fluxo direita-esquerda quando presente costuma acompanhar-se de alterações pressóricas na circulação pulmonar (por exemplo, embolia pulmonar maciça, hipertensão pulmonar primária), com hipertensão pulmonar e inversão do gradiente pressórico para direita-esquerda. Entretanto, alguns pacientes, como o aqui descrito, não apresentam aumento de pressão nas câmaras direitas, nem hipertensão pulmonar, fazendo com que o
Existe uma série de tentativas de explicar esse fenômeno5-7. Embora a pressão atrial média seja maior no átrio esquerdo do que no átrio direito, significativas alterações desse gradiente podem ocorrer no início da diástole e durante a contração isométrica do ventrículo direito. Essas alterações de gradiente podem ser magnificadas em situações como inspiração profunda, Valsalva, embolia pulmonar, hipoxemia, doença pulmonar obstrutiva crônica grave, infarto de ventrículo direito, pressão expiratória final positiva elevada em pacientes em posição sentada. Nesses casos, pode haver
O substrato anatômico para o
Além disso, a complacência das cavidades cardíacas direitas parece se reduzir com a idade podendo gerar um fluxo preferencial via comunicação interatrial. Outros fenômenos que parecem facilitar alterações no fluxo incluem distorções na anatomia das relações entre a veia cava inferior e o septo interatrial através de rotação do coração no sentido anti-horário e/ou desvio mediastinal, tendo como causas mais freqüentes pneumonectomia direita, derrame pericárdico e alterações na aorta ascendente7,8.
Outra abordagem que busca explicar as causas do fenômeno de
É fundamental que se busquem nesses substratos anatomofisiológicos e em conceitos embriológicos as origens desse fenômeno. Entretanto, a despeito desses conhecimentos, um completo entendimento dos mecanismos envolvidos na patogênese do
Embora os mecanismos envolvidos na patogênese não estejam completamente esclarecidos, parece haver um claro consenso em relação ao tratamento dessa situação clínica. Os relatos de caso1,2,8,9, bem como a maior serie disponível10 relatando tratamento desses pacientes incluem como ponto fundamental a correção do defeito interatrial, por via percutânea ou cirúrgica. Em nosso paciente, optou-se pela correção cirúrgica da comunicação interatrial, com excelente resultado.
A síndrome de platipnéia-ortodeoxia com
Thiago Lisboa Rua Carazinho, 559/100 90460-190 – Porto Alegre, RS E-mail:
Artigo recebido em 28/02/06; revisado recebido em 27/05/06; aceito em 08/08/06.
This article describes a patient presenting with dyspnea and platypnea and whose only clinical finding was presence of patent foramen ovale with a right to left shunt, without pulmonary hypertension, characteristic of the rare Platypnea-Orthodeoxya Syndrome, with very interesting pathophysiological findings and with therapeutic alternatives not yet defined.
Right to left shunt through a patent foramen ovale without pulmonary hypertension
Hospital de Clínicas de Porto Alegre - Porto Alegre, RS, Brazil
This paper describes a patient who presented with hypoxemia and platypnea and whose only finding on investigation was the presence of a patent foramen ovale with a right to left shunt, without pulmonary hypertension, the characteristic of the rare Platypnea-Orthodeoxia Syndrome which shows interesting physiopathological features. Therapeutic options have not yet been fully defined.
Cases of hypoxemia secondary to right to left shunt through a patent foramen ovale in the absence of pulmonary hypertension are rarely described in the literature and may constitute a picture of platypnea-orthodeoxia syndrome1. In this paper we will describe the case of a patient with this rare condition.
A 71-year old white male patient, married, was admitted to the Emergency Room of HCPA due to major dyspnea at rest, with no associated symptoms. He reported having progressive dyspnea for ten months, which became more intense ten days before admission.
His previous medical history included: smoking from 31 to 35 years of age; thoracic bruise with no sequela 25 years ago; systemic arterial hypertension since 1985, treated with Amlodipine, Hydrochlorothiazide and Enalapril; dyslipidemia since 1996, treated with Sinvastatin and gastroesophageal reflux disease treated with Omeprazole.
On physical examination, the patient was conscious, oriented, had tachypnea (RR=28 breaths/minute), and O2SAT= 88% on room air, in a seated position. He presented BP=140/80mmHg, HR=96bpm, with regular heart rhythm, with no extra sounds, normophonetic heart sounds, without murmurs. Peripheral pulse measurements of the four limbs were palpable and symmetrical; limb extremities were hot and cyanotic. He had no other alterations.
Tests showed: arterial blood gas analysis, on room air, with pH = 7.53; CO2P = 24.9; HCO3 = 20.3; O2P = 51.7; O2SAT= 90.5%; hematocrit = 50%, hemoglobin = 16.5 g/dl; normal white cell and platelet counts; normal heart enzymes; ECG without ischemic alterations; chest X-ray showing pleural calcifications; and chest angiotomography with no evidence of pulmonary thromboembolism.
He had progressive worsening of hypoxemia, and had to be admitted into the intensive care unit (ICU), at first with non-invasive mechanical ventilation and later with invasive mechanical ventilation, with two unsuccessful extubation attempts.
The investigation also included the following: pulmonary perfusion scintilography, with low probability of pulmonary thromboembolism; Doppler of lower limbs with no evidence of deep venous thrombosis; high resolution chest tomography showing no alterations that justified his degree of hypoxemia; transthoracic echocardiogram with air contrast, showing interatrial septal aneurysm, with intense and early passage of contrast to the left cavities, which is compatible with a patent foramen ovale, with no evidence of pulmonary arterial hypertension; enlarged left ventricle, with a relaxation deficit, and preserved global and segmental systolic function and with ejection fraction (EF) = 66%; transesophageal echocardiogram with Doppler, with evidences of a patent foramen ovale, with a predominant right to left flow, without intracavity thrombus; pulmonary arteriography with absence of intrapulmonary shunt.
With the presumptive diagnosis of hypoxemia secondary to the right to left shunt through the patent foramen ovale, we installed a pulmonary artery catheter and performed blood gas analysis. Later we performed cardiac catheterization and measured oxygen saturation in the heart chambers (
As the patient continued to present desaturation when in a seated position and therefore required mechanical ventilation despite the absence of a right left pressure gradient, he underwent surgical repair of the patent foramen ovale (atrioseptoplasty) (
This report describes a 71-year old male patient with a clinical picture of platypnea-orthodeoxia caused by right to left shunt through a patent foramen ovale with no increase in pulmonary circulation pressure.This is characteristic of a rare syndrome whose diagnosis depends on a high degree of suspicion, and that is frequently unrecognized. In this case, as in previous reports, the diagnosis was confirmed by a transesophageal echocardiography with contrast, with early passage of bubbles, demonstrating the presence of shunting and documenting the presence of interatrial communication. After this, we performed right and left cardiac catheterization which confirmed the presence of normal intracardiac pressures. Arterial oxygen saturation showed to be appropriate in this assessment and we believe this finding was due to the mechanical ventilation and to the patient’s decubitus, since he presented desaturation when standing, which is compatible with the picture of platypnea-orthodeoxia.
The cause of the right to left shunt with normal intracardiac pressures through an interatrial communication has not yet been completely clarified and, although very complex diagnostic tools are available, a series of questions remain unanswered as to the physiopathology and the anatomical and physiological substrate that can explain this picture1-3.
The clinical syndrome described as platypnea-orthodeoxia is very infrequent but is also overlooked; patients present dyspnea and hypoxemia associated with supine and seated body position, with improvement of symptoms when the patient lies down. This picture was first described by Burchell in 1949 and less than a hundred cases have been described since then4.
Under normal conditions, an interatrial communication allows left to right flow, due to the pressure gradient and to the greater complacency of the right chambers as compared with the left chambers. When a right to left flow exists there are usually pressure alterations in the pulmonary circulation (for instance, massive pulmonary embolism, primary pulmonary hypertension), with pulmonary hypertension and reversal of the pressure gradient to right-left. However, some patients, such as the one described here, do not present either a pressure increase in the right chambers or pulmonary hypertension, which causes shunting to be done against a pressure gradient4.
Several attempts have been made to explain this phenomenon5-7. Although the mean atrial pressure is higher in the left atrium than in the right atrium, significant alterations in this gradient may take place in the beginning of diastole and during right ventricle isometric contraction. These gradient alterations can be intensified in situations such as deep inspiration, Valsalva, pulmonary embolism, hypoxemia, severe chronic obstructive pulmonary disease, right ventricle infarction, elevated positive end-expiratory pressure in patients in a seated position. In these cases there may be right to left shunt in the presence of interatrial communication with atrial septum aneurysm and patent foramen ovale6.
The anatomical substrate for shunting with normal pressures includes, in addition to an interatrial communication that allows it to happen, a phenomenon of the flow7. This phenomenon would take place due to a preferential flow from the inferior vena cava towards the interatrial septum, as part of a remaining prenatal circulatory pattern.
In addition to this, the complacency of right cardiac cavities seems to decrease with age, which may generate a preferential flow through the interatrial communication. Other phenomena that seem to foster alterations in the flow include distortions in the anatomy of the relations between the inferior vena cava and the interatrial septum through the anticlockwise rotation of the heart and/or mediastinal deviation, which are more frequently caused by right pneumonectomy, pericardial effusion and alterations in the ascending aorta7,8.
Another approach that seeks to explain the causes of shunting relate to embryological aspects, and considers the atrial remodeling that takes place after the incorporation of the venous sinus to its structure and allows for independent flows from vena cava superior and vena cava inferior towards the interatrial septum. This could play a role in the origin of this phenomenon7.
It is essential to seek the origins of this phenomenon in these anatomical and physiological substrates and in embryological concepts. However, despite this knowledge, a full understanding of the mechanisms involved in the pathogenesis of right to left shunt with normal pressures has not yet been obtained.
Although the mechanisms involved in its pathogenesis have not yet been clarified, there seems to be clear consensus as to the treatment of this clinical condition. Case reports1,2,8,9, as well as the largest series available10 reporting on the treatment of these patients include the percutaneous or surgical repair of the interatrial communication as a key point. We decided to perform the surgical repair of our patient’s defect, and obtained an excellent result.
The platypnea-orthodeoxia syndrome, with right to left shunt with normal pressure levels is a rare and complex condition whose physiopathological mechanism has not yet been fully clarified. However, this diagnosis should be considered when there is hypoxemia and dyspnea, especially in patients in supine/seated position, since their treatment with the repair of the atrial septal defect that allows shunting presents excellent results as reported in the literature and as exemplified in this description.
Thiago Lisboa Rua Carazinho, 559/100 90460-190 – Porto Alegre, RS, Brazil E-mail:
Manuscript received February 28, 2006; revised manuscript received May 27, 2006; accepted August 8, 2006.