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Case 2/2014 - 20-year-old woman with corrected transposition, pulmonary atresia and aortopulmonary collateral arteries.

Atik E - Arq. Bras. Cardiol. (2014)

View Article: PubMed Central - PubMed

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The first one was characterized by dyspnea and tachypnea, frombirth to five months of age, increased pulmonary flow and heart failure, with cardiomegalyand hepatomegaly... The second phase was characterized by stability without significantsymptoms, which lasted until the age of three, presumptive of balanced aortopulmonary flow.The third phase, with decreased pulmonary flow, had mild cyanosis that worsened two yearsago... There wereatrioventricular and ventriculoarterial discordances, pulmonary atresia (PA) and largeventricular septal defect (VSD) in the 25 mm inflow... The pulmonary trunk was not viewed,neither were pulmonary arteries... Corrected transposition of the great arteries (CTGA),pulmonary atresia and ventricular septal defect in dextrocardia and situs solitus.Pulmonary circulation is nourished by aortopulmonary collateral arteries with pulmonaryarterial hypertension in severe hypoxia and natural evolution... Clinical findings of cyanogenic heart diseases,accompanied by PA + VSD may vary, often with signs of overflow, balancing flows ordecreased pulmonary blood flow, as noted in this patient under natural evolution... Theinitial overflow gave way others due to predominance of decreased pulmonary blood flow.In this patient, the last stage was due to increased pulmonary arterial hypertension,not diagnosed initially... Other clinical signs were indicative of CTGA: second heartsound in the pulmonary area, which increases to the lower right sternal edge, suggestingthat the aorta arises from the right ventricle to the left, backed by radiographicimaging, in which the ascending aorta is located on the left... The continuous murmur ofcollateral arteries expresses the presumed associated pulmonary atresia... This rare association, PA + VSD + CTGA and pulmonary circulationdependent on aortopulmonary collateral arteries was reported only once in theliterature... In general, the clinical picture depends on two defects (VSD + PA) guiding thepathophysiology of this set... When the collateral circulation is augmented with increasedpulmonary flow, it generally undergoes stabilization and subsequent decrease,characterizing the evolutionary stages... Hypoxemia arises from decreased pulmonary flowthrough stenosis of aortopulmonary circulation or the development of pulmonaryhypertension.

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Chest radiography shows the heart on the right in situs solitus, withcharacteristics, together with those of the electrocardiogram, correctedtransposition of the great arteries, with the left ventricle on the right and theaorta on the left.
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f01: Chest radiography shows the heart on the right in situs solitus, withcharacteristics, together with those of the electrocardiogram, correctedtransposition of the great arteries, with the left ventricle on the right and theaorta on the left.

Mentions: Electrocardiogram (Figure 1) showedjunctional rhythm and signs of left ventricular overload on the right. The P wave wasnegative in II, III, aVF and precordial areas. The QRS complex morphology was RS in V1,rs in V6 and Rs in V6R. The T wave was negative in I, L and more positive in V6R than inV6. AQRS: +160º, AT: 120º, AP: −80º.


Case 2/2014 - 20-year-old woman with corrected transposition, pulmonary atresia and aortopulmonary collateral arteries.

Atik E - Arq. Bras. Cardiol. (2014)

Chest radiography shows the heart on the right in situs solitus, withcharacteristics, together with those of the electrocardiogram, correctedtransposition of the great arteries, with the left ventricle on the right and theaorta on the left.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3987311&req=5

f01: Chest radiography shows the heart on the right in situs solitus, withcharacteristics, together with those of the electrocardiogram, correctedtransposition of the great arteries, with the left ventricle on the right and theaorta on the left.
Mentions: Electrocardiogram (Figure 1) showedjunctional rhythm and signs of left ventricular overload on the right. The P wave wasnegative in II, III, aVF and precordial areas. The QRS complex morphology was RS in V1,rs in V6 and Rs in V6R. The T wave was negative in I, L and more positive in V6R than inV6. AQRS: +160º, AT: 120º, AP: −80º.

View Article: PubMed Central - PubMed

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

The first one was characterized by dyspnea and tachypnea, frombirth to five months of age, increased pulmonary flow and heart failure, with cardiomegalyand hepatomegaly... The second phase was characterized by stability without significantsymptoms, which lasted until the age of three, presumptive of balanced aortopulmonary flow.The third phase, with decreased pulmonary flow, had mild cyanosis that worsened two yearsago... There wereatrioventricular and ventriculoarterial discordances, pulmonary atresia (PA) and largeventricular septal defect (VSD) in the 25 mm inflow... The pulmonary trunk was not viewed,neither were pulmonary arteries... Corrected transposition of the great arteries (CTGA),pulmonary atresia and ventricular septal defect in dextrocardia and situs solitus.Pulmonary circulation is nourished by aortopulmonary collateral arteries with pulmonaryarterial hypertension in severe hypoxia and natural evolution... Clinical findings of cyanogenic heart diseases,accompanied by PA + VSD may vary, often with signs of overflow, balancing flows ordecreased pulmonary blood flow, as noted in this patient under natural evolution... Theinitial overflow gave way others due to predominance of decreased pulmonary blood flow.In this patient, the last stage was due to increased pulmonary arterial hypertension,not diagnosed initially... Other clinical signs were indicative of CTGA: second heartsound in the pulmonary area, which increases to the lower right sternal edge, suggestingthat the aorta arises from the right ventricle to the left, backed by radiographicimaging, in which the ascending aorta is located on the left... The continuous murmur ofcollateral arteries expresses the presumed associated pulmonary atresia... This rare association, PA + VSD + CTGA and pulmonary circulationdependent on aortopulmonary collateral arteries was reported only once in theliterature... In general, the clinical picture depends on two defects (VSD + PA) guiding thepathophysiology of this set... When the collateral circulation is augmented with increasedpulmonary flow, it generally undergoes stabilization and subsequent decrease,characterizing the evolutionary stages... Hypoxemia arises from decreased pulmonary flowthrough stenosis of aortopulmonary circulation or the development of pulmonaryhypertension.

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