Pulmonary atresia with intact ventricular septum and right ventricular dependent coronary circulation through the “vessels of Wearn”

Ahmed, Atif; Snodgrass, Brett Thomas; Kaine, Stephen

doi:10.1016/j.carpath.2012.12.004

Cited by 12 publications

(17 citation statements)

References 32 publications

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“…For the purposes of extrapolating percentages, 40,000 CHDs per year are estimated . Repeat surgeries affect an estimated 6700–8700 children every year in the US, and cost the healthcare system hundreds of millions of dollars . The primary clinical application for an expandable conduit is most likely a nonvalved conduit.…”

Section: Discussionmentioning

confidence: 99%

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Mechanical and microstructural analysis of a radially expandable vascular conduit for neonatal and pediatric cardiovascular surgery

Loneker

Luketich

Bernstein³

et al. 2017

J Biomed Mater Res

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In pediatric cardiovascular surgery, there is a significant need for vascular prostheses that have the potential to grow with the patient following implantation. Current clinical options consist of nonexpanding conduits, requiring repeat surgeries as the patient outgrows the device. To address this issue, PECA Labs has developed a novel ePTFE vascular conduit with the capability of being radially expanded via balloon catheterization. In the described study, a systematic characterization and comparison of two proprietary ePTFE expandable conduits was conducted. Conduit sizes of 8 and 16 mm inner diameters for both conduits were evaluated before and after expansion with a 26 mm balloon. Comprehensive mechanical testing was completed, including quantification of circumferential, and longitudinal tensile strength, suture retention strength, burst strength, water entry pressure, dynamic compliance, and kink radius. Scanning electron microscopy was used to investigate the microstructural properties. Automated extraction of the fiber architectural features for each scanning electron micrograph was achieved with an algorithm for each conduit before and after expansion. Results showed that both conduits were able to expand significantly, to as much as 2.5× their original inner diameter. All mechanical properties were within clinically acceptable values following expansion. Analysis of the microstructure properties of the conduits revealed that the circumferential main angle of orientation, orientation index, and spatial periodicity did not significantly change following expansion, whereas the node area fraction decreased post expansion. Successful proof‐of‐concept of this novel product represents a critical step toward clinical translation and provides hope for newborns and growing children with congenital heart disease. © 2017 Wiley Periodicals, Inc. J Biomed Mater Res Part B: Appl Biomater, 106B: 659–671, 2018.

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Section: Discussionmentioning

confidence: 99%

“…The secondary market is a valved conduit for RVOTR. The annual US population for RVOTR was estimated at 3100 …”

Section: Discussionmentioning

confidence: 99%

Mechanical and microstructural analysis of a radially expandable vascular conduit for neonatal and pediatric cardiovascular surgery

Loneker

Luketich

Bernstein³

et al. 2017

J Biomed Mater Res

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“…Ventriculo‐coronary connections are common in PA‐IVS and may play a role in the poor outcome of these patients . The abnormal pulsatile perfusion of the coronary arteries with hypoxic desaturated blood at higher than systemic pressure may give rise to proliferative vasculitis . The coronaropathy is progressive and unpredictable leading to coronary artery obstruction, ischemia and lethal arrhythmias.…”

Section: Discussionmentioning

confidence: 99%

“…6 The suprasystemic systolic pressures wave induces changes such as dilatation of the sinusoids and intimal fibrodysplasia of the coronary arteries, frequently already during fetal life. [5][6][7][8] These may lead to retrograde perfusion of the coronary arteries with desaturated blood. Progressive intimal hyperplasia and medial hypertrophy follow and will progressively obstruct the lumen and reduce the vessel compliance, resulting in incapacity of the normal diastolic epicardial perfusion pressure to open the vessel and thereby requiring the suprasystemic pressure wave to provide flow though this coronary segment.…”

mentioning

confidence: 99%

Percutaneous obliteration of the right ventricle to avoid coronary damage by sinusoids in patients with pulmonary atresia intact ventricular septum during staged single ventricle palliation

Hubrechts¹,

Cools²,

Brown

et al. 2019

Cathet Cardio Intervent

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Background and Aims Suprasystemic pressure waves can damage the coronary arteries resulting in myocardial ischemia and excess early mortality. We aimed to reduce the coronary pressure wave through the sinusoids by abolishing RV volume with percutaneous devices. Methods and Results Four patients with PA‐IVS and coronary sinusoids from the hypertensive rudimentary RV were evaluated at a median age 26.6 months (range: 2.7–51.7). Right ventricle coronary dependent flow to the left ventricular myocardium was excluded. All four patients had dual perfusion with competitive flow from the RV through the sinusoids to the coronary arteries. Devices used were: Amplatzer vascular plug II of 10–16 mm; 27 coils (diameter 5–15 mm) in the oldest patient. Right ventricular angiography after cavity obliteration showed no more significant coronary perfusion through the sinusoids. There were no complications or deaths. Only minor and transient changes in the levels of troponin were observed. Coronary angiography at pre‐Fontan evaluation showed no progress of coronary abnormalities in two patients. Conclusion In selected patients with functionally single left ventricle, obliteration of the hypertensive RV cavity by percutaneous devices is safe and abolishes the systolic pressure wave in coronary sinusoids. When performed early, this may halt coronary damage and avoid excess mortality.

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“…Terms such as vessels of Wearn, Thebesians and coronary microfistulae are used interchangeably but erroneously, there being a defining anatomical nomenclature to which few adhere. Vessels of Wearn are known to be part of a cluster of cyanotic congenital heart defects surrounding pulmonary atresia with intact ventricular septum (PAIVS) 3. In PAIVS, they can coexist with atrial septal defect, tricuspid valve anomalies, persistent ductus arteriosus and proximal coronary anomalies such as ectasia, anomalous origin, stenosis, fibromuscular dysplasia and atresia 4.…”

Section: Discussionmentioning

confidence: 99%

Association of coronary to left ventricular microfistulae (vessels of Wearn) with atrial septal defect in an adult without cyanotic heart disease

Hussain¹,

Roberts

2015

BMJ Case Reports

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Vessels of Wearn are rare findings during coronary angiography in adults. They are known to be associated with forms of cyanotic congenital heart disease in infants but we are not aware of any published cases of association with non-cyanotic left to right shunts in adults. We present the case of a 69-year-old man with angiographically evident vessels of Wearn draining from the left and right coronary arteries into the left ventricle associated with an asymptomatic atrial septal defect. We postulate a developmental phase association between atrial septal maturation and closure of perfusing microchannels from the ventricular cavities to the epicardial coronary arteries on the same spectrum as that which leads to more widespread defects in infants. We also highlight a common medication side effect that might have been mistaken as a manifestation of the congenital anomalies.

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Pulmonary atresia with intact ventricular septum and right ventricular dependent coronary circulation through the “vessels of Wearn”

Cited by 12 publications

References 32 publications

Mechanical and microstructural analysis of a radially expandable vascular conduit for neonatal and pediatric cardiovascular surgery

Mechanical and microstructural analysis of a radially expandable vascular conduit for neonatal and pediatric cardiovascular surgery

Percutaneous obliteration of the right ventricle to avoid coronary damage by sinusoids in patients with pulmonary atresia intact ventricular septum during staged single ventricle palliation

Association of coronary to left ventricular microfistulae (vessels of Wearn) with atrial septal defect in an adult without cyanotic heart disease

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