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Atrial septal defect with normal pulmonary arterial pressure in adult cyanotic patient.

Kim IC, Kim H, Lee JE, Yoon HJ, Kim JB, Kim JH - J Cardiovasc Ultrasound (2014)

Bottom Line: A 22-year-old male presented with recurrent stroke, central cyanosis, and dyspnea.Transesophageal echocardiography and cardiac catheterization revealed bidirectional shunt flow through atrial septal defect (ASD) without pulmonary arterial hypertension.The orifice of inferior vena cava facing towards ASD opening led partially right to left shunt resulting in cyanosis with normal pulmonary arterial pressure.

View Article: PubMed Central - PubMed

Affiliation: Division of Cardiology, Department of Internal Medicine, Keimyung University Dongsan Medical Center, Daegu, Korea.

ABSTRACT
A 22-year-old male presented with recurrent stroke, central cyanosis, and dyspnea. Transesophageal echocardiography and cardiac catheterization revealed bidirectional shunt flow through atrial septal defect (ASD) without pulmonary arterial hypertension. The orifice of inferior vena cava facing towards ASD opening led partially right to left shunt resulting in cyanosis with normal pulmonary arterial pressure.

No MeSH data available.


Related in: MedlinePlus

Initial chest X-ray (A) and transthoracic echocardiography of parasternal long axis (B) and apical 4-chamber view (C). These tests showed that cardiac chambers were not enlarged and left ventricular ejection fraction was normal.
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Figure 2: Initial chest X-ray (A) and transthoracic echocardiography of parasternal long axis (B) and apical 4-chamber view (C). These tests showed that cardiac chambers were not enlarged and left ventricular ejection fraction was normal.

Mentions: On physical examination, clubbing fingers of both hands were detected (Fig. 1). Chest X-ray finding was normal with cardio-thoracic ratio of 0.4 (Fig. 2A). Electrocardiography revealed normal axis without evidence of cardiac chamber enlargement. Complete blood count showed polycythemia (hemoglobin 19.3 g/dL, hematocrit 56.4%) but JAK2 mutation was negative which suggests polycythemia was secondary change from hypoxemia. Brain magnetic resonance imaging (MRI) showed right cerebellar small embolic nature infarction. No intra- or extra-cranial vascular obstruction was detected. Interestingly, his initial blood gas analysis showed hypoxemia (PaO2 58.1 mm Hg, O2 saturation 90.7% at room air) with mild hypocapnea (PaCO2 27.3 mm Hg) which could not be corrected with oxygenation therapy (PaO2 70.8 mm Hg, O2 saturation 93.6% at 4 L oxygenation by nasal cannula). Putting the laboratory tests together, intra- or extra-cardiac shunt was suspected. However, on initial transthoracic echocardiography (TTE), there were no signs of heart chambers enlargement and no definite intra-cardiac shunt flow was detected (Fig. 2B and C). After the initial work-up, he was on clopidogrel 75 mg daily medication and discharged from neurology department without transesophageal echocardiography (TEE). After 6 months, he readmitted to neurology department with second attack of stroke. The brain MRI revealed acute embolic infarction on right thalamus without large vessel pathology. TTE with bubble test showed rapid filling of LA and left ventricle chambers with agitated bubble simultaneously within 3-4 cardiac cycles after filling right atrium (RA) and right ventricle chambers (Fig. 3, Supplementary movie 1). TEE showed ASD with bidirectional shunt flow (Fig. 4). Left to right shunt was dominant during most of the cardiac cycle, but right to left shunt was also visible during mid to late diastolic period. Heart dynamic computed tomography showed that the opening of IVC was facing toward ASD, guiding direct transmission to the LA via ASD (Fig. 5).


Atrial septal defect with normal pulmonary arterial pressure in adult cyanotic patient.

Kim IC, Kim H, Lee JE, Yoon HJ, Kim JB, Kim JH - J Cardiovasc Ultrasound (2014)

Initial chest X-ray (A) and transthoracic echocardiography of parasternal long axis (B) and apical 4-chamber view (C). These tests showed that cardiac chambers were not enlarged and left ventricular ejection fraction was normal.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4286645&req=5

Figure 2: Initial chest X-ray (A) and transthoracic echocardiography of parasternal long axis (B) and apical 4-chamber view (C). These tests showed that cardiac chambers were not enlarged and left ventricular ejection fraction was normal.
Mentions: On physical examination, clubbing fingers of both hands were detected (Fig. 1). Chest X-ray finding was normal with cardio-thoracic ratio of 0.4 (Fig. 2A). Electrocardiography revealed normal axis without evidence of cardiac chamber enlargement. Complete blood count showed polycythemia (hemoglobin 19.3 g/dL, hematocrit 56.4%) but JAK2 mutation was negative which suggests polycythemia was secondary change from hypoxemia. Brain magnetic resonance imaging (MRI) showed right cerebellar small embolic nature infarction. No intra- or extra-cranial vascular obstruction was detected. Interestingly, his initial blood gas analysis showed hypoxemia (PaO2 58.1 mm Hg, O2 saturation 90.7% at room air) with mild hypocapnea (PaCO2 27.3 mm Hg) which could not be corrected with oxygenation therapy (PaO2 70.8 mm Hg, O2 saturation 93.6% at 4 L oxygenation by nasal cannula). Putting the laboratory tests together, intra- or extra-cardiac shunt was suspected. However, on initial transthoracic echocardiography (TTE), there were no signs of heart chambers enlargement and no definite intra-cardiac shunt flow was detected (Fig. 2B and C). After the initial work-up, he was on clopidogrel 75 mg daily medication and discharged from neurology department without transesophageal echocardiography (TEE). After 6 months, he readmitted to neurology department with second attack of stroke. The brain MRI revealed acute embolic infarction on right thalamus without large vessel pathology. TTE with bubble test showed rapid filling of LA and left ventricle chambers with agitated bubble simultaneously within 3-4 cardiac cycles after filling right atrium (RA) and right ventricle chambers (Fig. 3, Supplementary movie 1). TEE showed ASD with bidirectional shunt flow (Fig. 4). Left to right shunt was dominant during most of the cardiac cycle, but right to left shunt was also visible during mid to late diastolic period. Heart dynamic computed tomography showed that the opening of IVC was facing toward ASD, guiding direct transmission to the LA via ASD (Fig. 5).

Bottom Line: A 22-year-old male presented with recurrent stroke, central cyanosis, and dyspnea.Transesophageal echocardiography and cardiac catheterization revealed bidirectional shunt flow through atrial septal defect (ASD) without pulmonary arterial hypertension.The orifice of inferior vena cava facing towards ASD opening led partially right to left shunt resulting in cyanosis with normal pulmonary arterial pressure.

View Article: PubMed Central - PubMed

Affiliation: Division of Cardiology, Department of Internal Medicine, Keimyung University Dongsan Medical Center, Daegu, Korea.

ABSTRACT
A 22-year-old male presented with recurrent stroke, central cyanosis, and dyspnea. Transesophageal echocardiography and cardiac catheterization revealed bidirectional shunt flow through atrial septal defect (ASD) without pulmonary arterial hypertension. The orifice of inferior vena cava facing towards ASD opening led partially right to left shunt resulting in cyanosis with normal pulmonary arterial pressure.

No MeSH data available.


Related in: MedlinePlus