Surgical Approach to Treatment Fetal period Dx- HLHS, intact atrial - - PowerPoint PPT Presentation

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Surgical Approach to Treatment

• f Pulmonary Vein Stenosis

Christopher Caldarone, MD 11th International Conference on Neonatal and Childhood Pulmonary Vascular Disease San Francisco 2018 04 21

Surgical – Good outcome

• Fetal period
• Dx- HLHS, intact atrial septum
• Pulmonary lymphangiectasia > stent
• Gradient in stent
• Delivered > O.R. (~2.4 kg)
• Resect atrial septum, stent, EFE
• Bilateral PAB/PGE
• 2 weeks
• Severe stenosis RLPV (MG 13)
• Probable involvement of RUPV/RLPV

RPV:LPV = 50%: 50% Qp(Pveins):Qs =1.26:1

Surgical – Good outcome

• 7 weeks
• Losartan
• Consider heart/lung transplant
• Surgical intervention (?)
• Problem
• Likely progressive PVS
• Single ventricle with high pulmonary flow
• Expect long waitlist
• Surgical objective
• Improve likelihood of surviving on waitlist for transplant

Focal stenosis Large upstream diameter

Surgical – Good outcome

• 8 weeks
• Sutureless repair
• Manage duct and bilateral PAB’s
• Extubated POD 5
• RUPV Pk:2mmHg
• RUPV Mn: 1mmHg
• RLPV Mn: 2mmHg
• LUPV Pk: 3mmHg
• LUPV Mn: 2mmHg

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Surgical – Good outcome

• 3 months

….No change

• PDA stented, transfer to home
• Expectation of recurrent disease
• Plan to list for transplant when recurrence evident

Surgical – Good outcome

• Favorable pulmonary vein anatomy

Surgical – Good outcome

7 months

• Norwood
• 4mm central BT shunt (Ab RSCA)
• Resection of RUPV and RLPV scars (?)
• Unobstructed pulmonary veins on postop TEE

Surgical – Good outcome (?)

• One year
• Qp/Qs = 0.6
• LPA small > stent
• Pulmonary veins OK
• 15 months
• Extensive pulmonary artery plasty
• BCPS
• Arch reconstruction with interposition
• Pulmonary veins OK
• Mediastinitis

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Learning points

• Upstream diameters are important
• Worst substrate
• Single ventricle - High flow pulmonary veins
• Fetal pulmonary venous hypertension
• But – large upstream vessels

Learning points

• Upstream diameters are important
• Worst substrate
• Single ventricle - High flow pulmonary veins
• Fetal pulmonary venous hypertension
• But – large upstream vessels

LoRito, JTCVS 2016

Learning points

• Sutureless repairs best for local disease and complex geometry

Learning points

• Sutureless repairs best for local disease and complex geometry
• Poor outcomes with congenital PVS

Viola, JTCVS 2011

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Learning points

• Sutureless repairs are best for local disease and complex geometry
• Good outcomes with pre-op obstruction
• n=768

Shi, Circulation 2017

Learning points

• Sutureless repairs best for local disease and complex geometry
• Good outcomes with mixed TAPV

Honjo, Ann Thos Surg 2010

Learning points

• Sutureless repairs best for local disease and complex geometry
• Post-repair PVS
• Kalfa – AATS 2017 – in press
• N=75, 14 centers
• PVS score risk adjustment
• Sutureless repair > less reoperation/reintervention

Kalfa, JTCVS, in press

Surgical – Poor outcome

• 2 months
• Cor triatriatum, Anomalous drainage LUPV
• O.R.
• Resection of the cor triatriatum membrane
• Sutureless repair of atrial junction with LLPV
• LUPV branch to VV to innominate
• 6 months postop
• RUPV pg 1; mg 0
• RLPV pg 4; mg 1
• LUPV to VV to inomminate vein; no obstruction
• LLPV pg 6; mg 2

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Surgical – Poor outcome

• 15 months postop
• LUPV to vertical vein and innominate vein
• LLPV severely narrowed
• Flow
• Anomalous LUPV: 2.1 L/min/m2
• LLPV: 0.18 L/min/m2
• Qp:Qs =1.67:1
• RPA:LPA = 63%:27%
• Cath lab, dilation, cutting balloon – minimal improvement

Surgical – Poor outcome

Good surgical substrate

Right atrium

Surgical – Poor outcome

• Options:
• No operation

Left Lung Left atrium 2.1 L/min/m2 0.2 L/min/m2 Right atrium

Surgical – Poor outcome

• Options:
• Repair LLPV and attach LUPV to LA

Left Lung Left atrium

RISK RISK

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Surgical – Poor outcome

• Options:
• Repair LLPV

RISK

Right atrium Left Lung Left atrium 2.1 L/min/m2

Surgical – Poor outcome

• One month postop
• LLPV pg 11mmHg
• LLPV mg 4mmHg
• 4 months postop
• Narrow/occluded LLPV
• Interstitial edema/lymphangiectasia
• Areas of segmental collapse or venous infarction

Learning points

Greenway , J Cardiovasc Mag Res 2011

• Flow redistribution
• Can be high volume!
• Introduces error in echo gradients
• False security
• Implications for surgical decisions

Learning points

• Competing flow can jeopardize an anastomosis

Chiu, Phys Rev, 2011 Right atrium Left Lung Left atrium 2.1 L/min/m2

RISK

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Learning points

• LUPV anastomosis can cause problems!

Kotani, Ann Thor Surg 2013

Don’t poke a skunk….

Sampling bias

• Small samples – weak evidence
• Not always representative
• Test a research question:
• Who is Henri Justino?
• Methodology:

Sampling bias

• Who is Henri Justino?

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Sampling bias

• Who is Henri Justino?

Is this representative of truth? Bearded? 1/6 >Not representative Further study required!

Sampling bias

• Who is Henry Justino?

Sampling bias

• Who is Henri Justino?

Is this representative of truth? Conclusions: Frequency of incarceration : 8/14 images Dr Justino is fond of orange jumpsuits Future study: What is Dr Justino doing in Hays County?

Balasubramanian, Circ 2012

Combined surgical/cath-based therapy

In-stent restenosis: 50% @ 1 year

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Balasubramanian, Circ 2012

Combined surgical/cath-based therapy

Better performance > 7mm

Balasubramanian, Circ 2012

Combined surgical/cath-based therapy

Survival: 50-60% @ 3 years

Lung transplant and PVS

5yr survival: 60% ECMO was main predictor of 1 year mortality

Bharat, J HeartLungTransplant 2013

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Sutureless repairs

Mavroudis / Pediatric Cardiac Surgery 5th Edition

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