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PH Professional Network: 2015

Nightmares in Pediatric PH

Russel Hirsch, M.D.

Director , Pulmonary Hypertension Service

The Heart Institute

Cincinnati Children’s Hospital Medical

Center

Cincinnati, Ohio

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Patricia Lawrence, PNP

Pediatric Nurse Practitioner

Children’s Healthcare of Atlanta

Atlanta. Georgia

Disclosures

Dr. Russel Hirsch:

• Proctor, St. Jude Medical

• Off-label use of approved drugs

Patricia Lawrence:

• Research support from Eli Lilly

• Off-label use of approved drugs

This continuing education activity is managed and accredited by

Professional Education Services Group in cooperation with the

Pulmonary Hypertension Association. Neither PESG, nor PHA, nor

any accrediting organization support or endorse any product or

service mentioned in the is activity.

PESG and PHA staff has no financial interest to disclose.

Commercial Support was not received for this activity.

Learning Objectives

At the conclusion of this activity, the participant will be able to:

1. Recognize risks associated with PH therapy and how PH therapy can

be harmful in certain patient populations

2. Describe solutions for preventing medication errors in the outpatient

setting for patients on parenteral PH treatments

3. Discuss potential side effects that impact a patient’s quality of life

Overview

• Every diagnosis of Idiopathic PH in a child is a

nightmare

• Cases we will discuss will highlight:– Difficulties in diagnosis

– Complexity of management

– Adverse effects of medications

– Impact of sepsis

– Autonomy and ethical aspects in decision making

KH, 12 year old female

• Asymptomatic, normal activities– Competitive basketball player

• Cleaning horse stall and kicked in the belly

• ER evaluation with severe abdominal pain /

hematuria

• MRI– Renal contusion

– Multiple hepatic lesions (AVM vs. hepatic nodules)

– Dilated IVC

KH: Initial Evaluation

• No cardiac symptoms

• BP 132/60

• Sternal heave / Palpable 2nd heart sound

• Loud, wide split S2

• 3cm hepatomegaly, mild tenderness

KH: Baseline Studies

KH: Baseline Echo Images

KH: Baseline MRI Images

KH: Baseline CC

BL

30%

FiO2

iNO 80ppm /

FiO2 80%

Rp

(WU x m2)

9.1 9.8

Rs

(WU x m2)

13.5

CI

(l/min/m2)

5.4 3.68

Mean PA

Pressure

(mmHg)

52 50

Mean RA

Pressure

(mmHg)

7

KH: Course

• Open liver biopsy during cardiac catheterization,

and hepatic vein injections– Focal nodular hyperplasia with malignant potential

– Portal venous malformation with Abernathy-type physiology

KH: Course

• Medications commenced as out-patient– Oral Sildenafil

– Inhaled treprostinil• Dose increased gradually to maximum of 9 breaths q4h

• Initial occasional dizziness, but then well tolerated

– Bosentan contra-indicated

• At 3 months– Asymptomatic

– Compliant

– Liver transplant evaluation in progress• Parents somewhat reticent at this stage

KH: Follow-Up Cath: 5 Wks

BL

30%

FiO2

Follow-Up

(5 weeks)

Rp

(WU x m2)

9.1 4.95

Rs

(WU x m2)

13.5 11.73

CI

(l/min/m2)

5.4 5.45

Mean PA

Pressure

(mmHg)

52 42

Mean RA

Pressure

(mmHg)

7 7

KH: Follow-Up: 9mo

• No change in symptoms

• Echo Deterioration– RV dilated / function worse

– Increased TR Doppler jet velocity

– Increased septal flattening

• Deterioration in hepatic parameters– Albumin decreased

– GGT increased

– Ammonia increased

KH: Follow-Up Cath: 10mo

BL

30%

FiO2

Follow-Up

(5 weeks)

Follow-Up

(10 mo)

Rp

(WU x m2)

9.1 4.95 7.8

Rs

(WU x m2)

13.5 11.73 8.0

CI

(l/min/m2)

5.4 5.45 4.6

Mean PA

Pressure

(mmHg)

52 42 52

Mean RA

Pressure

(mmHg)

7 7 9

KH: Clinical Adjustments

• Broviac central line placed– After much family discussion

• Converted from inhaled to IV treprostinil– Titrated up to a dose of 23ng/kg/min at time of DC

• Considerable more discussion in regard to liver

transplantation

Survival: Portopulmonary Hypertension

• REVEAL Registry data*• 5 yr survival 40% in subjects with PoPH versus 64% IPAH/FPAH

• Review of Mayo Data (1994-2007)**• 74 PoPH patients

• No treatment (19 pts): 5 yr survival 14%

• Pulm Vaso-dilator (43 pts): 5 yr survival 45%

• Liver Transplantation***• mPAP > 50mmHg - 100% liver transplant mortality

• mPAP < 35mmHg – 100% liver transplant survival

* Krowka et.al. Chest 2012;141:906-915

**Swanson et.al. AmJTransplant 2008;8:2445-2453

***Krowka et.al.Liver Transpl 2000;6:443-450

On-Going ManagementBL

30%

FiO2

Follow-

Up

(5

weeks)

Follow-

Up

(10 mo)

Follow-

Up

14 mo)

Rp

(WU x

m2)

9.1 4.95 7.8 6.59

Rs

(WU x

m2)

13.5 11.73 8.0 12.44

CI

(l/min/m2)

5.4 5.45 4.6 5.3

Mean PA

Pressure

(mmHg)

52 42 52 49

Mean RA

Pressure

(mmHg)

7 7 9 9

On-Going Management

• Regular clinic visits

• Further up-titration to 54ng/kg/min

• Generally feeling well, no complaints

• Full-time at school

• Intense conversation re liver transplantation

On-Going Management

BL

30%

FiO2

Follow-

Up

(5

weeks)

Follow-

Up

(10 mo)

Follow-

Up

14 mo)

Follow-

Up

(18 mo)

Rp

(WU x m2)

9.1 4.95 7.8 6.59 6.1

Rs

(WU x m2)

13.5 11.73 8.0 12.44

CI

(l/min/m2)

5.4 5.45 4.6 5.3 6.1

Mean PA

Pressure

(mmHg)

52 42 52 49 51

Mean RA

Pressure

(mmHg)

7 7 9 9 9

Clinical Course

• Presented to local ER 4 weeks post cath– Low grade fever x 2 days

– Subtle loss of appetite

– Diagnosed with UTI, treated nitrofurantoin

– Fever resolved

• Called PH office 3 weeks later– 7.5Kg weight loss

– Intermittent diarrhea

– Malaise

– Febrile

Acute Presentation

• Unwell appearing

• Bloated

• Pale

• Febrile to 38.7°C

• Pulse 128/min

• BP 135/80

• RR 20/min

Acute Presentation

• Mild peripheral edema

• Hyperdynamic precordium

• Prominent sternal heave / palpable P2

• Loud S2 with, with wide split P2

• 3/6 holosystolic murmur at tricuspid area

• Liver unchanged / no ascites

• Clear lung fields

CXR

First Presentation Current Presentation

New Studies

New Study

First Presentation Current Presentation

New Studies

Presumed Diagnosis: Endocarditis

• Blood Cultures– Positive for Streptococcus Mitis (CVC as well as peripheral cultures)

• CRP: 10.9 (< 0.3)/ ESR: > 40

• CBC / chemistries / liver enzymes stable

• Mild increase in INR

Subsequent Course

• Commenced on Ceftriaxone

• Over course of 6 day stay– Initial mild clinical improvement

– Broviac removed / PICC line inserted once cultures negative

– Fever resolved

– Appetite initially improved, then deteriorated

• Just prior to discharge– Sleeping more

– Mild peripheral edema

– Further loss of appetite

– Echo deterioration

Echo Prior to Discharge

Options……

• Repair / replace the tricuspid valve– Almost certain RV failure with need for mechanical support

? Endpoint

• Repair / replace the tricuspid valve with

transition to Novalung? Endpoint

• Repair / replace the tricuspid valve with

transition to Novalung, and list for lung and liver

transplant

The Family’s Decision

• To go home…..– Neurologically and behaviorally intact teenager

– Previously perfectly well

– Curable PH with liver transplant with excellent prognosis

– No part in decision making

– Patient was not consulted

– Parents did not wish to have her informed of the issues or the prognosis

– Parents did not wish to have palliative care involved

Course

• Discharged on stable medications– IV Treprostinil 54ng/kg/min via PICC

– Sildenafil

– Lasix

– Antibiotics (to complete 6 week course)

– Analgesia

– Nasal cannula oxygen

• Over first 2-3 weeks– Shortness of breath

– Peripheral edema

– Bloated belly

– Intermittent chest pain

– Increased sleepiness and lethargy

Course

• Activities– Family road trip to Montreal

– Make-a-Wish to Hawaii to swim with dolphins• (needed to be supported in the water)

• Completed antibiotics 5 weeks post discharge

• Family decided to forgo follow-up appointment at

that time

………..then……

• 8 weeks post discharge, mother called to report

that she seemed to be doing better

Follow-Up Visit at 10 Weeks

• Felt well, no complaints

• Normal appetite

• No shortness of breath

• No further swelling of her legs or belly bloating

• No longer using supplemental O2

• No analgesia

• Wanted to go back to school

• Resumed riding her horse

Follow-Up Visit at 10 Weeks

• Exam:– P 90/min; BP 120/70; O2 Sat 100%

– Peripheries warm / normal pulses

– Quite precordium

– Mild prominence of the S2, with variable split

– Quiet 1/6 holosystolic murmur at the left upper sternal border

– Clear lung fields

– Soft belly; no masses

Follow-Up Echo

Follow Up Echo

Wow!!!!!!!

Where to from here….?

• Scheduled for cath…….rescheduled

• Maintained on same medication

• Credibility of care givers a major family issue

• Pathophysiology of her course is remarkable

• Teenager patient autonomy


Case #2


CS, a 10 year old male with

an unusual history

• Presented to our PH team in December 2012 after admission to PICU for hypoxia and shortness of breath

• Cardiology fellow saw patient night of admission

• “He’s had a normal echo in the past, but let’s just do another one”

• Echo findings led to PH inpatient consult


Chart review and detailed history

2011

Syncope

2/2011

Nl head CT

ref to

Cardiology

Normal

EKG & Echo

March

2011

Normal

exercise test

March 2011

2011

Syncope

2/2011

Nl head CT

ref to

Cardiology

Normal

EKG & Echo

March

2011

Syncope

4/25/2011

Borderline

QTc

-Cardiology

Consult-

Event

Syncope

4/30/2011

with event

monitor

Syncope

5/7/2011Tilt

table

Admission



• Referral and visits to Neurology on 5/2011 and

8/2011

• Exam and EEGs all suggestive of neurocardiogenic

syncope

• Felt to have ‘reactive autonomic nervous system’

2011

1

2

3

4

Syncope # 5

11/2011



• 2/2012 - Referral to endocrinology

– No endocrinologic explanations for syncopal episodes

2011 2012

5/2012 11/2012 Admission

5 Syncopal

Episodes

2011 2012

5/2012 11/2012

5 Syncopal

Episodes

12/7/12 Admission


CS meets the PH team after 12/7/12 admission

for hypoxia


CS meets the PH team after 12/7/12 admission

for hypoxia

Tests included:

• Walk test distance 378, desaturation to 85%

• PSG without OSA, + nocturnal hypoxemia

• Pulmonary Function Tests


12/2012 Admission

CXR

• CXR - interstitial lung

disease vs pulmonary

hemorrhage


12/2012 Admission

Chest CT


Abnormal vs Normal Chest CT


12/2012 Admission

Echocardiogram


PH “Crisis”

• After getting emotional & angry with parents after

learning he may not go home for a few days


12/2012 Admission

cardiac catheterization

Rest iNO + O2

CVP 11

mPAp 52

PVR 15.9 9.1

Wedge 10

CI

• Attempted bronch in cath lab, discontinued

• Extensive pruning of distal small branches

• Started on tadalafil with no evidence

of pulmonary edema

Rest iNO + O2

CVP 11 11

mPAp 52 43

PVR 15.9 9.1

Wedge 10 11

CI 2.6 3.5


Several PH “crises” at home

• 12/2012 and 1/2013 requiring short admissions

• Usually occurred after emotional outburst or activity

• Saturations would often dip to mid 70s

• lasix started, tadalafil stopped and restarted at smaller dose


Lung biopsy

• 1/29/2013- Confirmed

PVOD

– Arrest after

procedure requiring

intubation, pressors

– Pulse steroids started


Photos of lung surface

at time of biopsy


Photos of lung surface

at time of biopsy


Normal lung surface


Pulmonary Veno-occlusive Disease

Normal Abnormal


PVOD

• Overlap of PVOD and PCH

• Causes of PVOD unknown

• 5-10% of cases diagnosed with idiopathic PAH

• Wide age range but primarily in children and adults

• Median survival ~ 2 years from diagnosis

• Symptoms include cough, hypoxia and progressive

dyspnea


Pediatric PVOD – a retrospective analysis


PH clinic 4/2013

• Echo

• Walk test

• PH staff witnessed crisis immediately after walk

test


PH crisis in clinic after walk

• Sats to 70s

• HR to 140

• Flash pulmonary

edema with crackles

which resolved within

1 hour


Admission after PH crisis 4/2013

• Admission – started Imatinib

– PACT team consult

– Family not interested in lung transplant

– Hospice

– Patient not consulted about wishes (11 years old at time)


Imatinib for PVOD


Clinical improvement within weeks

CT one year later


Chest CT comparison


Echocardiogram at time of repeat Chest CT


Subsequent visits

• Stable echos

• Complaint of ‘breathing bothers me’

• Steroid weans

• Main complaint of abdominal pain

• Constipation


Interim Data

Red – TR jet

Gold –walk

Blue - BNP


Follow up cardiac catheterization

12/2012 1/2015

CVP 11 9

mPAp 52 48

PVR 15.9 11.7

Wedge 10 11

CI 2.6 3.14

-Increased tadalafil

-Later started calcium channel blocker


Subsequent visits

• Occasional complaints of chest pain and difficulty with

breathing but no change in vital signs, echo

• No syncopal episodes since 12/2012 since starting on

tadalafil

• No PH crises from 4/2013 when Imatinib started

• Patient not informed of diagnosis or prognosis


And then...a PH crisis

April 2015

• Increased CCB

• Another PACT team visit

• Steroid burst


PH clinic 7/22/15

• Seen for complaints of chest tightness, increased

oxygen requirement

• Echo stable

• BNP less than 10

• Normal physical exam with splitting of S2

• Long discussion about family’s wishes given ‘return’ of

PH crises


And then…..a syncopal episode

Admitted 8/13/15


Clinical Data

• BNP normal

• LFTs normal

• CXR improved 12 hours later

• O2 requirement returned to normal


Most recent clinical data

Red – BNP

Gold –TR jet

Blue - PVR


8/13/15 Admission for syncopal episode

• Family devastated by ‘return’ of syncope

• Imatinib increased

• Discontinued amlodipine

• Palliative care team involvement

• Discussions around lung transplant resumed

• Family shared diagnosis and prognosis with patient


Hot off the presses

• Clinic visit 9/16/15

• TR 120

• 6 minute walk distance 308

• BNP 70

• Admitted to restart amlodipine, monitor

• ? Intercurrent illness?

• Family has appointment with lung transplant center for

evaluation


What next?

• Additional PAH therapy?

• Atrial Septostomy?

• Lung transplant?

2011 2012 2013 2014 2015

5 Syncopal

Episodes

3 Syncopal

Episodes

1 Admit 4 Admits 5 Admits

1 Syncopal

Episode

1 Admit 3 Admit


Pulmonary Hypertension Team

at CHOA

• Amanda Brown, PNP

• Anna Burnett Gay, PNP

• Jeryl Huckaby, RRT

• Usama Kanaan, MD

• Nikhil Chanani, MD

• Dawn Simon, MD

Pulmonary Hypertension Team

at

CCHCM

• Michelle Cash

• Alison Cress

• Lisa Burns

• Jenna Faircloth

• Kathy Gosney

• Bill Nichols

• Katie Lutz

• Mike Pauciulo

• The nurses on A6C and

in the CICU and NICU

• Fellows who assist with

consultations


What questions do you have?


Obtaining CME/CE Credit

If you would like to receive continuing education

credit for this activity, please visit:

http://pha.cds.pesgce.com

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