Giant Left Atrium Associated With Mitral Valve Prolapse

Cheng, Tsung O.

doi:10.1161/01.cir.0000018751.52074.e9

Cited by 17 publications

(6 citation statements)

References 5 publications

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“…All patients were more than 50 years old, with the medium age in the 7th decade, which fits the theory that most of the subtle anatomic changes present in the cases with right-to-left shunt are age-related, which could explain the late onset of symptoms [22]. Aging may affect the size of the PFO as it is related to aortic root dilation, kyphoscoliosis, distortion of the interatrial septum or stiffness of the right ventricle.…”

Section: Resultssupporting

confidence: 62%

“…As Cheng [22] has stated, we believe that, besides the structural component provided by the existence of an interatrial communication, a functional premise also has to be present to cause this inverted direction of flow with orthostatism.…”

Section: Introductionmentioning

confidence: 90%

“…About 10 years ago, only 40 cases of POS had been described [19], and by 2009, about 130 patients with platypnea and orthodeoxia had been identified [20,21], but without a clear distinction of the causes that had led to it [22]. …”

Section: Introductionmentioning

confidence: 99%

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Platypnea-Orthodeoxia Syndrome in Review: Defining a New Disease?

Rodrigues¹,

Palma²,

Sousa-Pereira³

2012

Cardiology

127

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Platypnea-orthodeoxia syndrome is an intriguing condition that comprehends multiple pathologic phenomena. It requires a high clinical suspicion and is probably underdiagnosed. Its identification has therapeutic implications and can have a huge impact on the patient’s everyday life. We made a review of the literature and analyzed 188 cases described thus far, trying to identify the underlying mechanisms and risk factors. We propose that a separate entity is created within the spectrum of platypnea-orthodeoxia syndrome that is responsible for the majority of cases, namely platypnea-orthodeoxia disease, which would be defined as the existence of an interatrial communication, with a right-to-left shunt, normal pressure in the right atrium and, of course, platypnea and orthodeoxia. Most patients with this disease were submitted to percutaneous closure of the patent foramen ovale or atrial septal defect, with favorable results, making it the first-line therapeutic approach nowadays.

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Section: Resultssupporting

confidence: 62%

Section: Introductionmentioning

confidence: 90%

See 1 more Smart Citation

Platypnea-Orthodeoxia Syndrome in Review: Defining a New Disease?

Rodrigues¹,

Palma²,

Sousa-Pereira³

2012

Cardiology

127

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“…However, the presence of only these anatomical factors cannot induce POS because they usually cause left-to-right shunting. In addition, functional factors, such as right hemidiaphragmatic paralysis, pericardial effusion, constrictive pericarditis, emphysema, arteriovenous malformation, pneumonectomy, liver cirrhosis, aortic aneurysm, or elongation changes of shunt flow can determine POS [7]. Many previous reports indicated that POS can occur for various reasons.…”

Section: Discussionmentioning

confidence: 99%

Platypnea-orthodeoxia syndrome in the right lateral decubitus position: a case report

et al. 2017

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BackgroundPlatypnea-orthodeoxia syndrome is a rare syndrome characterized by dyspnea and hypoxia when the patient is sitting or standing. Here we report a case of platypnea-orthodeoxia syndrome caused by a right hemidiaphragmatic elevation with giant liver cyst that triggered a right-to-left shunt through the patent foramen ovale. This case report is the first presentation of a case secondary to hemidiaphragmatic elevation with giant liver cyst. In addition to this, a malposition of the pacemaker lead could be associated with platypnea-orthodeoxia syndrome in this case.Case presentationA 91-year-old Japanese woman presented to our hospital with hypoxia of unknown origin. Severe hypoxia and cyanosis were observed only in the right lateral decubitus position. A chest X-ray and computed tomography scan revealed right hemidiaphragmatic elevation, which was probably compressing the right atrium. A transesophageal echocardiogram showed a compressed right atrium and shunt blood flow in both directions: from the left to the right atrium and vice versa. The shunt flow was exacerbated by postural changes from the left to the right lateral decubitus. A transesophageal echocardiogram also confirmed compression of the right atrium due to giant liver cyst and a malposition of the pacemaker lead abnormally placed in the left atrium through patent foramen ovale. We concluded that the cause of hypoxia was platypnea-orthodeoxia syndrome with right-to-left interatrial shunt through patent foramen ovale. Surgical closure of patent foramen ovale was not performed due to the age of our patient, surgical difficulties, and failure to obtain informed consent. For these reasons she was discharged after receiving medical advice about her posture.ConclusionsPlatypnea-orthodeoxia syndrome is rare and difficult to diagnose. The present case suggests that hypoxia due to postural changes should be considered a differential diagnosis of platypnea-orthodeoxia syndrome.

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“…However, its rarity in the current era is particularly due to the decreased incidence of rheumatic fever. A giant LA of non-rheumatic origin is, instead, extremely unusual when caused by severe mitral regurgitation accompanying mitral valve prolapse [4]. However, a giant LA of non-rheumatic origin is a rare entity because it is not presumably associated with primary disease of the left atrial myocardium, in which the remodeling of the musculature is implicated in the face of rheumatic heart disease and with a higher incidence in mitral valve disease.…”

mentioning

confidence: 99%

Giant left atrium in a woman with mitral prosthetic valve malfunction and history of rheumatic heart disease

et al. 2009

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A 65-year-old woman with a past medical history of rheumatic heart disease, hypertension, chronic atrial fibrillation and chronic obstructive pulmonary disease was admitted to our department with a chief complaint of cough and shortness of breath worsened in the last month. She had undergone mechanical mitral valve prosthesis replacement for severe mitral regurgitation when she was 42 years old, in 1982. In the emergency department (ED), a chest X-ray study showed a marked prominence of the right cardiac border, nearly complete opacification of the lower lung fields and splaying of the carina ( Fig. 1). At the time of admission, she was on treatment with diuretics, digoxin and acenocoumarol. There were no complaints of voice hoarseness, dysphagia or any other gastrointestinal symptoms. The hemogasanalysis was normal. An EKG showed atrial fibrillation. The trans-thoracic echocardiography (Table 1) revealed a mild decrease of systolic function at rest (ejection fraction of 46%), and moderate stenosis and regurgitation of the aortic valve, with mean and maximum gradients of 18.8 and 30.5 mmHg and a valve area of 0.97 cm 2 . This examination also unexpectedly demonstrated a massively enlarged left atrium (LA), greater than the left and right ventricles, with a maximum diameter of 13 cm and a transverse diameter of 11.8 cm (104 cm 2 ) on the apical four-chamber view. Roughly calculating the atrium as a sphere, we reached a volume of 1 litre (Fig. 2). There was an associated moderate to severe regurgitation of the mitral valve prosthesis with a trans-prosthetic mean and maximum gradients of 8.45 and 19.28 mmHg, and a valve area of 1.08 cm 2 . There was also evidence of dilated right-side heart sections, moderate tricuspidal regurgitation and pulmonary artery systolic pressure of 60 mmHg. Laboratory examinations revealed normal renal and liver functions with mild hyponatremia (132 mEq/L) and a mild iron deficiency anemia (Hb 11.6 mg/dl). No evidence of active rheumatic disease was documented. The ultrasound study of the abdomen showed congestive hepatomegaly. A diagnosis of decompensated heart failure syndrome (NYHA class III) was made, and oral treatment with high doses of furosemide, spironolactone and angiotensinreceptor blockers was initiated to manage the symptoms.During the hospitalization, because of the occurrence of bronchostenosis and leukocytosis, it was necessary to begin a course of treatment with steroids in infusion and an aerosol as well. After discharge, she was referred to the cardio-thoracic center of our hospital to evaluate the possibility of mitral valve prosthesis replacement.Left atrial enlargement is frequently found in clinical practice in a variety of heart conditions including rheumatic or non-rheumatic valvular heart disease, left ventricular diastolic dysfunction, hypertension, obesity, lone atrial fibrillation and left-to-right shunts [1]. Nevertheless, a giant LA is uncommon and defined according to the X-ray study appearance in which the LA forms the right border of the heart shadow and ...

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Giant Left Atrium Associated With Mitral Valve Prolapse

Cited by 17 publications

References 5 publications

Platypnea-Orthodeoxia Syndrome in Review: Defining a New Disease?

Platypnea-Orthodeoxia Syndrome in Review: Defining a New Disease?

Platypnea-orthodeoxia syndrome in the right lateral decubitus position: a case report

Giant left atrium in a woman with mitral prosthetic valve malfunction and history of rheumatic heart disease

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