Literature DB >> 29440849

Improving outcomes in totally anomalous systemic venous connection.

Sivasankaran Sivasubramonian1, Arun Gopalakrishnan1, Deepa Sasikumar1, K M Krishnamoorthy1.   

Abstract

Entities:  

Year:  2018        PMID: 29440849      PMCID: PMC5803970          DOI: 10.4103/apc.APC_133_17

Source DB:  PubMed          Journal:  Ann Pediatr Cardiol        ISSN: 0974-5149


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Sir, We congratulate the authors for their elegant description and case-based illustration of totally anomalous systemic venous connection (TASVC).[1] We would like to present a few points that the authors could clarify. The concept of connection and the physiology of drainage are well illustrated. We would like to extend the concept of venoatrial concordance here, in that the systemic venous drainage in left isomerism is always discordant venoatrial connection, but can be physiologically concordant by its drainage into pulmonary arteries from the right side. Then, the systemic veins draining into left-sided atrium have both physiological and anatomical discordance and major surgical implications. The failure of the right atrium and the tricuspid annulus to grow in TASVC is understandably related to the preload deprivation as explained by the authors. Is it not possible that the process of altered growth may not be entirely limited to the intrauterine period? In such a scenario, the natural history of these patients could potentially be altered by early diagnosis and venous rerouting surgical options early in infancy, thereby rendering the patient a candidate for a one-and-half or biventricular repair later in life. An analogous situation would be the infant who presents with a large atrial septal defect (ASD) with torrential left-to-right shunt augmented by mildly hypoplastic left ventricular inflow. In this example, the left heart tends to grow after ASD closure early in infancy.[2] We also appreciate the authors to have achieved good success after bidirectional Glenn surgery in the first patient with elevated pulmonary artery mean pressures and high pulmonary vascular resistance at the age of 13 years. We would like to know the outcome of this child. Could the natural history of the patient have been better if she had presented early in infancy? It is good to know that both nature and surgeons are revising the limitations of creating a Fontan circuit so that current limitation of age and pulmonary vascular resistance is revised to higher numbers.[34]

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Conflicts of interest

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  4 in total

1.  Fontan "Ten Commandments" revisited and revised.

Authors:  Herbert J Stern
Journal:  Pediatr Cardiol       Date:  2010-10-20       Impact factor: 1.655

2.  Conditions with right ventricular pressure and volume overload, and a small left ventricle: "hypoplastic" left ventricle or simply a squashed ventricle?

Authors:  C K Phoon; N H Silverman
Journal:  J Am Coll Cardiol       Date:  1997-11-15       Impact factor: 24.094

Review 3.  Clarifying the anatomy and physiology of totally anomalous systemic venous connection.

Authors:  Saurabh Kumar Gupta; Rajnish Juneja; Robert H Anderson; Gurpreet S Gulati; Velayoudam Devagorou
Journal:  Ann Pediatr Cardiol       Date:  2017 Sep-Dec

4.  Acquired Fontan paradox in isolated right ventricular cardiomyopathy.

Authors:  Mahim Saran; Sivasankaran Sivasubramonian; Sreevilasam P Abhilash; Jaganmohan A Tharakan
Journal:  Ann Pediatr Cardiol       Date:  2016 Sep-Dec
  4 in total

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