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Posts Tagged ‘pulmonary atresia with vsd’

Pulmonary atresia with VSD : Is it a cono-truncal anomaly ?

Posted in Uncategorized, tagged , , , , , , on August 26, 2025|

There is a list of conditions that come under cono-truncal anomaly(CTA) in CHD. TOF is the classical example. CTA are group of genetically determined fusion defects between developing right ventricle , septum and the outflows .Many components of outflow are outsourced from cranially located neural crest . In fact, improper migration and fusion of this secondary heart field to the primary is most common cause of many complex heart disease. Microdeletion on chromosome 22, specifically in the 22q11.2 is a major documented gene defect. Traditionally CTA list often includes PA with VSD. This table compares the anatomical and embryological basis of TOF & PA with VSD. You can decide whether to call both as part of cono-truncal anomaly or not.

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One important difference is missing in the above table. It is about presence and absence of PDA. In TOF true PDA may be present in early days or months . In PA with VSD , PDA or (MAPCAS mimicking a PDA ) can be part of collateral .A persistent controversy is, how to differentiate a PDA from MAPCAS .That can be challenging. Further, to label a vessel as PDA it should drain into normally formed left pulmonary artery, which is rarely likely.

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Pulmonary atresia with VSD : Does routine uni-focalisation strategy justify all the trouble ?

Posted in Uncategorized, tagged , , on February 9, 2020|

Pulmonary atresia with VSD is one of the complex CHD subsets that requires a meticulous understanding of anatomy, physiology of pulmonary circulation. It can be termed as TAPAC -Total anomalous pulmonary arterial connection in extreme cases. Should we attempt to reverse this total chaotic pulmonary blood supply is the question?

It demands a highly focused cath study(hands & brain) and CT Imaging which might actually throw more light. Post-study Interaction with surgeon and team of cardiologist are vital. The decision to take up the challenge of surgery or abandoning poses equal intellectual stress. Continuous and critical decisions need to be taken. Repeat surgeries and cath based Interventions are often a rule.  Very few centers have mastered this surgery.

A single slide presentation

 

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In spite of all technological developments in pediatric cardiac surgery, there is considerable variations and expectation of the surgical outcome. The major surprise is the original Melbourne group(Ref 1 )  that advocated the uni-focalization as a  concept has almost abandoned this. Stanford and other groups still continue to use this technique more often as a single-stage procedure to improve the outcome.

Let us hope these children get the best of the right mix of technology and natural survival power and more importantly we must ensure the former do not interfere with the later

Reference 

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Surgery has definitely  revolutionized the outcome in neonates and children in less severe forms of PA with good central pulmonary arteries ( Most of the Barbero Marcial Type A and many type B) The perceived negativity in this post regarding the outcome of surgery is primarily belong to some of the  Barbero Type B and many of  C.

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Barbero-Marcial M , Jatene A Semin Thorac Cardiovasc Surg. Surgical management of the anomalies of the pulmonary arteries in the tetralogy of Fallot with pulmonary atresia.1990 Jan;2(1):93-107. 

 

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