A staged decompression of right ventricle allows growth of right ventricle and subsequent biventricular repair in patients with pulmonary atresia and intact ventricular septum

Kotani, Yasuhiro; Kasahara, Shingo; Fujii, Yasuhiro; Eitoku, Takahiro; Baba, Kenji; Otsuki, Shinichi; Kuroko, Yosuke; Arai, Sadahiko; Sano, Shunji

doi:10.1093/ejcts/ezw124

Cited by 30 publications

(36 citation statements)

References 21 publications

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“…This staged approach should not be seen as a failure of the initial procedure, but rather as the tailoring of treatment to fit such a heterogeneous group. 7 Re-intervention rates also include additional work on the ductus stent, such as re-dilation and eventual percutaneous occlusion.…”

Section: Discussionmentioning

confidence: 99%

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A low threshold for neonatal intervention yields a high rate of biventricular outcomes in pulmonary atresia with intact ventricular septum

et al. 2020

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Aims:Management strategies for pulmonary atresia with intact ventricular septum are variable and are based on right ventricular morphology and associated abnormalities. Catheter perforation of the pulmonary valve provides an alternative strategy to surgery in the neonatal period. We sought to assess the long-term outcome in terms of survival, re-intervention, and functional ventricular outcome in the setting of a 26-year single-centre experience of low threshold inclusion criteria for percutaneous valvotomy.Methods and results:Retrospective analysis of patients diagnosed with pulmonary atresia with intact ventricular septum from 1990 to 2016 at a tertiary referral centre, was performed. Of 71 patients, 48 were brought to the catheterisation laboratory for intervention. Catheter valvotomy was successful in 45 patients (94%). Twenty-three patients (51%) also underwent ductus arteriosus stenting. The length of intensive care and hospital stay was significantly shorter, and early re-interventions were significantly reduced in the catheterisation group. There were eight deaths (17%); all within 35 days of the procedure. Of the survivors, only one has required a Fontan circulation. Twenty-eight patients (74%) have undergone biventricular repair and nine patients (24%) have one-and-a-half ventricle circulation. Following successful valvotomy, 80% of patients required further catheter-based or surgical interventions.Conclusions:A low threshold for initial interventional management yielded a high rate of successful biventricular circulations. Although mortality was low in patients who survived the peri-procedural period, the rate of re-intervention remained high in all groups.

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Section: Discussionmentioning

confidence: 99%

“…Many patients require multiple surgical procedures in their lifetime, depending on the growth and performance of the ventricle. 6,7 Catheter perforation of pulmonary valve in this condition was pioneered at our institution over 25 years ago. 8 It is now an established intervention and the procedure of choice in those with suitable anatomy.…”

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confidence: 99%

A low threshold for neonatal intervention yields a high rate of biventricular outcomes in pulmonary atresia with intact ventricular septum

et al. 2020

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“…This subgroup with coronary sinusoids has typically small hypoplastic ventricles with a single ventricle strategy being the only option . In patients with coronary artery stenosis or RVDCC to the left ventricular myocardium, decompression of the right ventricle may involve myocardial infarction with fatal outcome—special care should be taken during surgery to maintain coronary perfusion .…”

Section: Discussionmentioning

confidence: 99%

“…This subgroup with coronary sinusoids has typically small hypoplastic ventricles with a single ventricle strategy being the only option. 13 In patients with coronary artery stenosis or RVDCC to the left ventricular myocardium, decompression of the right ventricle may involve myocardial infarction with fatal outcome-special care should be taken during surgery to maintain coronary perfusion. 8,14 Limited treatment options with varying degrees of success have been reported for this subgroup and consist of right ventricle plication, tricuspid valve narrowing or closure, or ligation of ventriculo-coronary connections; when the LV is significantly compromised, cardiac transplant may be the only option.…”

Section: Discussionmentioning

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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“…While some reports suggest that this capacity is limited to growth proportional to somatic growth, 23,24 others suggest that there is capacity for growth beyond the matching of somatic growth, in even the smallest of RVs. [25][26][27] However, anatomic and physiologic factors associated with RV growth have not been extensively investigated.…”

Section: Discussionmentioning

confidence: 99%

Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study

Maskatia

Petit

Travers

et al. 2018

Congenital Heart Disease

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Background In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echocardiographic parameters associated with 2V circulation, (2) identify echocardiographic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions. Methods We performed a retrospective analysis of patients who underwent RV decompression for PA/IVS at four centers. We analyzed echocardiograms at baseline, postdecompression, and at follow up (closest to 1‐year or prior to Glenn circulation). Results Eighty‐one patients were included. At last follow‐up, 70 (86%) patients had 2V circulations, 7 (9%) had 1.5 ventricle circulations, and 4 (5%) had single ventricle circulations. Follow‐up echocardiograms were available in 43 (53%) patients. The majority of patients had improved RV systolic function, less tricuspid regurgitation (TR), and more left‐to‐right atrial shunting at a median of 350 days after decompression. Multivariable analysis demonstrated that larger baseline tricuspid valve (TV) z‐score (P = .017), ≥ moderate baseline TR (P = .045) and smaller baseline RV area (P < .001) were associated with larger increases in RV area. Baseline RV area ≥6 cm2/m2 had 93% sensitivity and 80% specificity for identifying patients who ultimately achieved 2V circulation. All patients with RV area ≥8 cm2/m2 at follow up achieved 2V circulation. This finding was confirmed in a validation cohort from a separate center (N = 25). Factors associated with achieving RV area ≥8 cm2/m2 included larger TV z‐score (P = .004), ≥ moderate baseline TR (P = .031), and ≥ moderate postdecompression pulmonary regurgitation (P = .002). Conclusions Patients with PA/IVS and smaller TV annuli are at risk for poor RV growth. Volume‐loading conditions signal increased capacity for growth sufficient for 2V circulation.

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A staged decompression of right ventricle allows growth of right ventricle and subsequent biventricular repair in patients with pulmonary atresia and intact ventricular septum

Cited by 30 publications

References 21 publications

A low threshold for neonatal intervention yields a high rate of biventricular outcomes in pulmonary atresia with intact ventricular septum

A low threshold for neonatal intervention yields a high rate of biventricular outcomes in pulmonary atresia with intact ventricular septum

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

Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study

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