Late emergence of platypnea orthodeoxia: Chiari network and atrial septal hypertrophy demonstrated with transoesophageal echocardiography

Platypnoea‐orthodeoxia is an infrequent syndrome that is usually associated with positional intracardiac right‐to‐left shunting. The authors report the case of a patient who presented with deteriorating severe dyspnoea and deoxygenation in the upright position that was partially relieved by recumbent repositioning. Dynamic echocardiography provided insight into the underlying anatomical and pathophysiological mechanisms as well as guidance for definitive therapy.

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“…Stretch of the interatrial communication, prominent Eustachian valve and thoracic aortic aneurysm 6 have been linked to the anatomical changes 7 …”

Section: Discussionmentioning

confidence: 99%

Dynamic echocardiography in evaluation of platypnoea‐orthodeoxia

Yastrebov

Sader

Youssef

et al. 2012

Australas J Ultrason Med

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“…Persistent Eustachian valve or the presence of Chiari’s network, which are embryonic remnants of the right valve of the sinus venosus [48,49], have also been described in POS patients and may potentiate direction of flow from the inferior vena cava to the atrial communication. Lipomatous hypertrophy of the inferior portion of the atrial septum, deforming the septum, has also been suggested to promote right-to-left shunting [49,50].…”

Section: Introductionmentioning

confidence: 99%

“…Lipomatous hypertrophy of the inferior portion of the atrial septum, deforming the septum, has also been suggested to promote right-to-left shunting [49,50]. …”

Section: Introductionmentioning

confidence: 99%

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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“…Catheters [21], guidewires [22], and pacemaker leads [23] may become entrapped within the network during invasive procedures. CN has also been demonstrated in association with neurofibromatosis [24], Behçet syndrome [25], platypnea-orthodeoxia [26], and migraine [27] and therefore it is not always a benign structure [28].…”

Section: Discussionmentioning

confidence: 99%

Untitled

2017

J Clin Anal Med

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Özet Amaç: Bu çalışma ile pediyatrik kardiyoloji polikliniğine müracaat eden çocuklardaki Chiari Ağı'nın (CA) transtorasik ekokardiyografi ile prevalansını değerlendirmesi ve diğer kardiyak anomalilerle birlikteliğinin belirlenmesi amaçlanmıştır. Gereç ve Yöntem: Hastanemizin çocuk kardiyoloji bölümünde Nisan 2013-Nisan 2014 yılları arasında kardiyak muayene ve transtorasik ekokardiyografi uygulanan 2232 çocuk çalışmaya alındı. Rutin M-mode, 2-D ve Doppler uygulandı. Tüm eşlik eden lezyonlar kaydedildi. Erken ve geç diyastolik akım hızı zirve yüksekliklerinin (E/A) 1'in altında olması muhtemel sağ ve sol ventriküler diyastolik disfonksiyonu düşündürdü. Bulgular: 2232 çocuktan 76'sında CA tespit edildi (%3.4) ve en sık ilk bir ayda saptandı. CA ile birlikte en sık görülen anomaliler atrial septal defect (ASD) ve patent foramen ovale (PFO) idi (%55.3). 10 hastada (%13.2) periferik pulmoner stenoz (PPS) ve 7 hastada (%9.21) atriyal septal anevrizma (ASA) tespit edildi. 2232 hastanın 267'sinde (%8.4) ASA saptanırken CA'lı hastalarda ASA sadece 7 kişide (%9.6) saptandı. Tartışma: CA sık görülmeyen, tesadüfi olarak tespit edilebilen ve doğru bir şekilde tanısı konması gereken, her zaman masum olmayan bir defekttir. Bu her zaman normal bir varyant olmayabilir. İlerde olabilecek muhtemel komplikasyonlar nedeniyle Chiari ağı uygun bir şekilde dökümente edilmelidir. AbstractAim: The study was performed to assess the prevalence of Chiari's Network (CN) in a pediatric outpatient department population by using transthoracic echocardiography (TTE) and to determine its association with other cardiac anomalies. Material and Method: 2232 children who underwent cardiac examination and TTE at the pediatric cardiology outpatient department of our institution between April 2013 and April 2014 were included in the study. Routine M-mode, 2-D, and Doppler studies were applied. All co-existent lesions were recorded. Ratio between heights of early and late diastolic flow velocity peaks (E/A) were <1 in tricuspid and mitral valves considered as possible right and left diastolic dysfunctions respectively .(Bu cümle eklendi.) Results: Of 2232 children, CN was detected in 76 patients (3.41%) and found most frequently during the first month. Atrial septal defect and patent foramen ovale (ASD/PFO) was the most common congenital defect seen with CN (55.3%). Ten children (13.2%) had peripheral pulmonary stenosis (PPS) and seven (9.21%) showed atrial septal aneurism (ASA) associated with CN. Of patients with CN, a total of 11 patients (14.5%), 10 of them 1-8 days old and 1 of them 17 days old, had E/A<1 in tricuspid valve. Three (3.9%) patients 1-12 days old demonstrated E/A<1 in tricuspid and mitral valves. Twenty-nine of the studied patients (11%) demonstrated E/A<1 in tricuspid valve (90% 1-30 days old). Of the total 2232 patients, 267 individuals (8.4%) showed ASA whereas there were only 7 cases (9.6%) of CN patients. Discussion: CN is an uncommon and incidental finding that should be recognized appropriately. This may not be always a normal v...

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Late emergence of platypnea orthodeoxia: Chiari network and atrial septal hypertrophy demonstrated with transoesophageal echocardiography

Cited by 18 publications

References 3 publications

Dynamic echocardiography in evaluation of platypnoea‐orthodeoxia

Dynamic echocardiography in evaluation of platypnoea‐orthodeoxia

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

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