Palliation of Cardiac Malformations Associated with Right Isomerism (Asplenia Syndrome) in Infancy

Donato, Roberto Di; Carlo, Duccio Di; Squitieri, C.; Rossi, Eugenio; Ammirati, Antonio; Marino, Bruno; Marcelletti, Carlo

doi:10.1016/s0003-4975(10)62353-3

Cited by 53 publications

(23 citation statements)

References 16 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Non-confluent pulmonary arteries had a strong association with mortality [11]. Similar results have been reported by other authors [12][13][14], with higher mortality figures for RI. These groups have highlighted the association of TAPVC with single ventricle as an important risk factor for mortality, especially if there is pre-operative pulmonary venous obstruction [15].…”

Section: Most Of the Descriptions In Current Literature Are Based On supporting

confidence: 87%

“…The relative lack of obstructed TAPVC in our series could again be attributed to the late age at presentation, the more sick neonatal forms having succumbed earlier on. Other authors have reported adverse surgical outcomes in heterotaxy with TAPVC particularly in neonatal period [12,17]. AV septal defect is a common substrate in visceral heterotaxy, more so in RI.…”

Section: Most Of the Descriptions In Current Literature Are Based On mentioning

confidence: 97%

See 1 more Smart Citation

Visceral Heterotaxy in the Developing World

Changlani

Kotecha

Changlani

et al. 2012

Heart, Lung and Circulation

View full text Add to dashboard Cite

Section: Most Of the Descriptions In Current Literature Are Based On supporting

confidence: 87%

Section: Most Of the Descriptions In Current Literature Are Based On mentioning

confidence: 97%

Visceral Heterotaxy in the Developing World

Changlani

Kotecha

Changlani

et al. 2012

Heart, Lung and Circulation

View full text Add to dashboard Cite

“…Early total intracardiac repair may not be advisable because of the complexity of the lesion. 9 If obstruction to the pulmonary venous return is present, nonetheless, aggressive and early repair is recommended. 810 Lamberti and his colleagues 11 suggested simultaneous construction of a systemic-to-pulmonary artery shunt and relief of pulmonary venous obstruction without cardiopulmonary bypass, but again this seems to be feasible with a reasonable rate of success only in older children.…”

Section: Discussionmentioning

confidence: 99%

“…Alternatively a systemic-to-pulmonary artery shunt of a smaller size must be planned prior to surgery. 9 Our experience has shown that excessive flow through a systemic-to-pulmonary artery shunt can be reduced by partial occlusion of its lumen by means of inflation of a balloon catheter. The same catheter can also be used to confirm the diagnosis of obstruction to the pulmonary venous return by pulmonary angiography.…”

Section: Discussionmentioning

confidence: 99%

Concealed pulmonary venous obstruction in right atrial isomerism with pulmonary outflow tract obstruction—surgical management following modified Blalock-Taussig shunt

Chiu

Wang

et al. 1992

Cardiol Young

View full text Add to dashboard Cite

Obstruction to the pulmonary venous return is a frequent associated anomaly in patients with isomerism of the right atrial appendages. Yet, preoperative diagnosis by means of either cross-sectional echocardiography or cardiac catheterization can be intriguing. Indeed, the presence of two morphologically right lungs reduce considerably the size of window for precordial echocardiography. Also, in the presence of severe pulmonary stenosis or atresia, it can be difficult at cardiac catheterization to enter the pulmonary trunk. In these patients, construction of a systemicto-pulmonary artery anastomosis will almost inevitably result in pulmonary edema. Between May 1984 and December 1988, five patients with isomerism of the right atrial appendages, severely decreased pulmonary blood flow and concealed obstruction to the pulmonary venous return were admitted to our hospital. A modified BlalockTaussig shunt by interposition of a polytetrafluoroethylene prosthesis was performed in each patients and all of them developed pulmonary edema. Three patients died despite appropriate medical treatment. The remaining two patients were successfully treated by banding of the Blalock shunt. This was performed in the first patient at the time of the initial surgery, when prior to closure of chest, pulmonary edema became manifest. The second patient who developed pulmonary edema early postoperatively, underwent cardiac catheterization to confirm the clinical diagnosis of obstruction to the pulmonary venous return. Reduction of blood flow through the Blalock shunt with resolution of edema was initially achieved by means of a partially occluding balloon catheter. Pulmonary edema recurred one week later because of rupture of the balloon and the patient eventually underwent a successful banding of the Blalock shunt through a left thoracotomy. We conclude that preoperative assessment of the pulmonary venous return is mandatory in patients with right isomerism and reduced pulmonary blood flow. Construction of a modified Blalock-Taussig shunt in the presence of concealed obstruction to the pulmonary venous return will almost inevitably cause pulmonary edema. Banding of the Blalock shunt can be successful, as observed in our experience, for the management of this serious complication.

show abstract

“…Auch in neueren Studien verstarben 53% dieser Patienten bereits in der Neonatalperiode vor oder während der initialen Palliation [10,13,16,24]. Selbst ohne klinisch nachweisbare pulmonalvenöse Obstruktion muss häufig von einer abnormen pulmonalvenösen Gefäßwandtex-tur ausgegangen werden.…”

Section: Biventrikuläre Korrekturoperationunclassified

Atriale Isomerie und viszerale Heterotaxie

Kaulitz

Ziemer

Hofbeck

2004

Herz

View full text Add to dashboard Cite

Atrial isomerism and visceral heterotaxy describe complex pathoanatomic findings with defects in the determination of lateralization. Differentiation of right and left atrial isomerism was based on the anatomy of the atrial appendages and bronchial tree and often associated with asplenia in right atrial isomerism and polysplenia in left atrial isomerism. In these pa tients complex cardiac anomalies determine clinical symptoms and diagnostic procedures. At primary diagnosis in the neonatal period, systemic venous and pulmonary venous connections and intracardiac anatomy are identified using the sequential segmental approach. Therapeutic decisions and palliative procedures can usually be based on these initial echocardiographic findings. As many of the patients were found to have a functionally univentricular heart, surgical procedures following the Fontan principle (lateral tunnel or extracardiac conduit procedure) were introduced in patients with heterotaxy syndrome and successfully performed. Early survival and long-term outcome vary depending on associated cardiac and systemic and pulmonary venous anomalies. The therapeutic options and mid-term results in patients with heterotaxy syndrome undergoing the Fontan procedure are reported.

show abstract

Palliation of Cardiac Malformations Associated with Right Isomerism (Asplenia Syndrome) in Infancy

Cited by 53 publications

References 16 publications

Visceral Heterotaxy in the Developing World

Visceral Heterotaxy in the Developing World

Concealed pulmonary venous obstruction in right atrial isomerism with pulmonary outflow tract obstruction—surgical management following modified Blalock-Taussig shunt

Atriale Isomerie und viszerale Heterotaxie

Contact Info

Product

Resources

About