Abstract 15398: Standing Breathless: A Case of Platypnea-Orthodeoxia Syndrome

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Adam S Vohra ◽  
Pritha Subramanyam ◽  
Harsimran S Singh
Keyword(s):  
Lung Metastases ◽  
Multimodality Imaging ◽  
Metastatic Cancer ◽  
Supplemental Oxygen ◽  
Right Heart Catheterization ◽  
Physical Exam ◽  
Heart Catheterization ◽  
Atrial Septal Aneurysm ◽  
Exertional Dyspnea ◽  
History Of

A 67-year old women with a history of metastatic endometrial cancer, prior pulmonary embolism (PE) (on Xarelto) and mitral valve prolapse presented for 2-3 weeks of exertional dyspnea. Patient presented to oncology clinic with oxygen saturation (SpO2) in the mid-70% on room air requiring supplemental oxygen, and eventually high flow nasal cannula (HFNC). She was transferred to our cardiac intensive care unit for further management of her hypoxia. Physical exam was notable for decreasing SpO2 from supine (99%) to sitting (88%) to standing (79%). In this patient with metastatic cancer, differential diagnosis for hypoxia was broad including pneumonia, metastatic pulmonary disease, doxorubicin-induced cardiomyopathy, and PE. CT chest showed no signs of pulmonary edema, pneumonia, lung metastases, or PE. Transthoracic echocardiogram showed normal left and right ventricular function with moderate aortic regurgitation and mitral regurgitation. However, agitated saline injection showed right to left flow consistent with intraatrial shunt. Transesophageal echocardiogram confirmed large patent foramen ovale (PFO) with atrial septal aneurysm. Given physical examination findings, presence of PFO, and structural abnormalities of intraatrial septum, platypnea-orthodeoxia syndrome (POS) was considered. Right heart catheterization was completed to evaluate shunt physiology and showed unremarkable pressures (RA 1, RV 22/1, PCWP 3, PA 20/3, and LA 2). Venous oxygen saturations did not show any step-up suggesting left to right shunt. Exercise challenge did not result in any significant change in PA or PCWP pressures. Given persistent concern for POS without other clear diagnosis, patient underwent percutaneous closure of PFO with a 30mm Gore Cardioform septal occluder. Patient had resolution of her exertional hypoxia following procedure and was discharged home the next day. The case discusses 1) the importance of history of and physical exam in narrowing diagnosis, 2) utilization of multimodality imaging to evaluate intraatrial shunts, and 3) use of percutaneous methods for PFO closure.

THE NATURAL HISTORY OF PULMONARY HYPERTENSION IN CHILDREN WITH VENTRICULAR SEPTAL DEFECTS ASSESSED BY SERIAL RIGHT-HEART CATHETERIZATION

PEDIATRICS ◽  
1961 ◽  
Vol 27 (4) ◽  
pp. 621-626
Author(s):  
Robert E. Stanton ◽  
Donald C. Fyler
Keyword(s):  
Natural History ◽  
Arterial Pressure ◽  
Pulmonary Arterial Pressure ◽  
Clinical Status ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Ventricular Septal Defects ◽  
Septal Defects ◽  
History Of ◽  
Pulmonary Arterial

The natural history of the pulmonary arterial pressure in 23 children with isolated ventricular septal defects has been evaluated by serial night heart catheterization. In none of the 23 patients was there a significant increase in pulmonary arterial pressure. Eight patients showed a significant decrease. The direction of shunts did not change between procedures, and the clinical status was stable. Our experience correlated well with the majority of institutions. It would appear that the natural course of pulmonary arterial pressure in ventricular septal defect is not commonly one of progressive rise.

scholarly journals Seizures in Idiopathic Pulmonary Arterial Hypertension

2018 ◽  
Vol 05 (02) ◽  
pp. 107-109
Author(s):  
Jamir Pitton Rissardo ◽  
Ana Letícia Fornari Caprara
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Supplemental Oxygen ◽  
Right Heart Catheterization ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Heart Catheterization ◽  
Right Heart ◽  
Systemic Disorder ◽  
Pulmonary Arterial

AbstractPulmonary arterial hypertension (PAH) is a progressive pulmonary vasculopathy. A 29-year-old female patient presenting with dyspnea and syncope within 6 hours of onset was admitted to our hospital. The patient stated that she looked for a neurologist months ago because she experienced abrupt shaking limbs occurring during physical activity. She was diagnosed with focal seizure, and carbamazepine (CBZ) was started. On admission, she reported that the dyspnea had started in the last week and recurrent episodes of syncope in the last few hours. A right heart catheterization was diagnostic of PAH. She was started on spironolactone, furosemide, sildenafil, warfarin, and supplemental oxygen. On 10th admission day, the patient was seizure free and the dose of CBZ was tapered. In the follow-up, the patient remained seizure free. An investigation to search for a chronic lung disease or hypoxemia, systemic disorder, hematological disorder, and metabolic disorder was negative.

Syncope in a Child with Pulmonary Hypertension and Positive Gene Tests for Hereditary Hemorrhagic Telangiectasia and Long QT Syndrome

2020 ◽  
Vol 18 (1) ◽  
pp. 70-76
Author(s):  
Bibhuti B. Das ◽  
Kak-Chen Chan
Keyword(s):  
Pulmonary Hypertension ◽  
Long Qt Syndrome ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Pulmonary Angiography ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Long Qt ◽  
Ct Angiogram ◽  
History Of ◽  
Qt Syndrome

We present a 10-year-old boy with syncope who was found to have long-QT syndrome and severe Pulmonary Hypertension (PH) both in the absence of a secondary cause; to our knowledge, this is the first report with this unusual coexistence. His genetic tests were positive for hereditary hemorrhagic telangiectasia and Long QT Syndrome (LQTS) without any family history of PH or LQTS. We demonstrated that digital subtraction pulmonary angiography was more useful compared to CT angiogram to demonstrate pulmonary vascular changes which correlated with a noresponse to acute vasoreactivity testing during right heart catheterization. He has been stable for the last 2 years on Ambrisentan, Sildenafil, and Nadolol without recurrence of symptoms.

scholarly journals Isolated Native Tricuspid Valve Endocarditis Caused by Viridans Streptococcus

2001 ◽  
Vol 12 (5) ◽  
pp. 305-307 ◽  
Author(s):  
John Swiston ◽  
Stephen D Shafran ◽  
Narmin Kassam
Keyword(s):  
Tricuspid Valve ◽  
Present Report ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Intravenous Drug Use ◽  
Right Heart ◽  
First Case ◽  
History Of ◽  
Viridans Group Streptococcus ◽  
Tricuspid Valve Endocarditis

The present report describes a case of native tricuspid valve endocarditis caused by viridans group streptococcus in a 43-year-old man who had recently undergone dental extraction. The patient had no history of intravenous drug use, heart disease or right heart catheterization. Although there have been scattered reports of unusual organisms, to the authors' knowledge, this is the first case of viridans group streptococcal endocarditis involving only the tricuspid valve after dental manipulation.

Thalassemia Associated Pulmonary Hypertension

2020 ◽  
Author(s):  
Mozhgan Hashemieh ◽  
Kourosh Sheibani
Keyword(s):  
Pulmonary Hypertension ◽  
Screening Method ◽  
Premature Death ◽  
Thalassemia Major ◽  
Right Heart Catheterization ◽  
Diagnostic Methods ◽  
Heart Catheterization ◽  
History Of ◽  
Receptor Blockers ◽  
Accumulation History

Cardiac disease is the main cause of death in both forms of thalassemia; thalassemia major (TM) and thalassemia intermedia (TI). Pulmonary hypertension (PH) is one of the cardiopulmonary morbidities with high mortality that, if not treated, may trigger right-sided heart failure and premature death. PH is defined as a mean pulmonary artery pressure of ≥25 mmHg at rest or ≥30 mmHg during exercise. The prevalence of PH is known to be higher in TI than in TM. Moreover, the pathophysiology of PH in thalassemia appears to be sophisticated and complex. Risk factors for occurrence of PH consists of non-transfusion dependent thalassemia (NTDT), sub-optimally transfused transfusion dependent thalassemia (TDT), splenectomy, thrombocytosis, anemia, NRBC ≥ 300 × 106, iron accumulation, history of thrombosis and older age. Other parameters which aggravate the risk of PH include hemolysis, oxidative stress, hypoxemia, alteration of erythrocyte membrane, decline of nitric oxide biological availability, arginine abnormal regulation and arginase excess. The screening method for PH is Doppler echocardiography but the gold standard for detection of PH is right heart catheterization (RHC). Current medical therapeutic options in PH comprise hydroxyurea, L- Carnitine, sildenafil, calcium channel antagonists, endothelin 1-receptor blockers and prostacyclin agonists. The only curative surgical method for the refractory and severe cases of PH is pulmonary endarterectomy. In this article, the etiology, pathophysiology, diagnostic methods and novel therapies of thalassemia associated PH are discussed.

scholarly journals History of Right Heart Catheterization

2010 ◽  
Vol 18 (2) ◽  
pp. 94-101 ◽  
Author(s):  
Bobby D. Nossaman ◽  
Brittni A. Scruggs ◽  
Vaughn E. Nossaman ◽  
Subramanyam N. Murthy ◽  
Philip J. Kadowitz
Keyword(s):  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
History Of

scholarly journals A rare case of quadrileaflet mitral valve and ostium primum atrial septal defect

2021 ◽  
Author(s):  
Corrado Fiore ◽  
Tugba Kemaloglu Oz ◽  
Luigi Lombardi ◽  
Rebani Sinani ◽  
Renato Gregorini ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Atrial Septal Defect ◽  
Rare Case ◽  
Septal Defect ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Progressive Dyspnea ◽  
History Of ◽  
Asd Closure

A 60-year-old female was referred to our clinic for evaluation of her rapidly progressive dyspnea, she had no previuos history of heart disease. A murmur was noted on her examination and transthoracic echocardiography was so difficult to be performed due to poor acoustic windows so she was referred to do a transesophageal echocardiography that showed an ostium primum atrial septal defect (ASD) with left to right shunt and a quadrileaflet mitral valve with severe regurgitation. Later on, she underwent surgery with Ostium Primum ASD closure by a patch and double cleft repair by suture after right heart catheterization.

scholarly journals P641 Severe pulmonary steno-insufficiency due to metastatic carcinoid tumor in a 57 years old patient treated with MTPV implantation: first experience

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
G M Viani ◽  
V L Paiocchi ◽  
L A Leo ◽  
F F Faletra ◽  
G Pedrazzini
Keyword(s):  
Carcinoid Tumor ◽  
Carcinoid Syndrome ◽  
Pulmonary Valve ◽  
Multimodality Imaging ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Heart Team ◽  
Right Heart ◽  
Metastatic Carcinoid ◽  
Melody Valve

Abstract Funding Acknowledgements none Background Right side heart valve involvement is a well known phenomenon in metastatic carcinoid disease. In recent times bioprosthetic valves have been widely used for replacement of stenotic and regurgitant native heart valves. Melody Transcatheter Pulmonary Valve (MTPV, Medtronic, Minneapolis, MN, USA) has been approved for treatment of native pulmonary valves as well as failing biological prostheses and conduits. We present the first case to our knowledge of severe native pulmonary valve steno-insufficiency due to metastatic carcinoid syndrome treated with MTPV implantation. Case report A 57-year-old woman is known since 2007 for metastatic carcinoid syndrome treated with surgical resection followed by chemotherapy (tamoxifene, everolimus) and radiotherapy. For 2 years she has been known for tricuspid and pulmonary involvement, which aggravated progressively, leading to symptomatic right heart failure. She was therefore submitted for multimodality imaging evaluation (see picture) and right heart catheterization. Transthoracic echocardiogram, cardiac MRI and right heart catheterization confirmed severe pulmonary valve steno-insufficiency. Cardiopulmonary exercise testing revealed a severe limitation of aerobic exercise capacity (VO2 max 14.4 ml/min/kg). After heart team discussion it was decided to treat the patient in a 2 step approach, initially with implantation of a Melody valve in pulmonary position and subsequently with surgical repair of the tricuspid valve. Pulmonary valve was treated by placement of a 39 x 25 mm ANDRA stent and subsequent implantation of a 22 mm Melody valve. Before stenting, coronary angiography with simultaneous balloon inflation in RVOT was performed to exclude coronary artery compression. During the procedure the patient developed hypotension, which was treated effectively with Octeotride. Post-operative echocardiogram showed a significant reduction in pulmonary valve gradient and no significant residual regurgitation. Conclusions We treated effectively severe pulmonary steno-insufficiency due to metastatic carcinoid tumor with MTPV implantation. No complications occurred. Multimodality imaging is fundamental for patients selection and preoperative planning of prosthetic valve dimensions. Abstract P641 Figure. Multimodality imaging of PV

Acute hemodynamic effects of NO and sildenafil during right heart catheterization in PAH and nonPAH-PH

Pneumologie ◽  
2015 ◽  
Vol 69 (05) ◽  
Author(s):  
V Foris ◽  
G Kovacs ◽  
P Douschan ◽  
X Kqiku ◽  
C Hesse ◽  
...  
Keyword(s):  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Hemodynamic Effects ◽  
Right Heart

Correlation of Data Obtained by Radiocardiography and Right-Heart Catheterization in Cardiac Patients

1968 ◽  
Vol 07 (02) ◽  
pp. 125-129
Author(s):  
J. Měštan ◽  
V. Aschenbrenner ◽  
A. Michaljanič
Keyword(s):  
Capillary Pressure ◽  
Transit Time ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Capillary ◽  
Filling Time ◽  
Pulmonary Capillary Pressure ◽  
The Mean ◽  
Pulmonary Transit Time

SummaryIn patients with acquired and congenital valvular heart disease correlations of the parameters of the radiocardiographic curve (filling time of the right heart, minimal pulmonary transit time, peak-to-peak pulmonary transit time, and the so-called filling time of the left heart) with the mean pulmonary artery pressure and the mean pulmonary “capillary” pressure were studied. Further, a regression equation was determined by means of which the mean pulmonary “capillary” pressure can be predicted.

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