MANAGEMENT OF LATE PRESENTATION OF CONGENITAL HEART DESEASE

Guillermo E. Moreno

Pediatric Cardiac Intensive Care Unit (UCI35)

Hospital de Pediatría “Dr. Juan P. Garrahan”

Buenos Aires - Argentina

• Non financial disclosure.

LATE PRESENTATION OF CHD

ARGENTINA Population 43. 590. 368 hab. (2016)

Area 3.745.997 km2

Population Density

10,7 Hab/ Km2

Healthcare system

Public: 85% Private: 15%

LATE PRESENTATION OF CHD

1492 km

3092 km

1002 KM

Difficult Access: 35%

Hospital de Pediatría “Dr. Juan P. Garrahan”

CICU beds 21

MSICU 48 beds BICU 12 beds

60

CICU Admissions 669

Admitted Surgical Cases

580 (14% neonates)

CPB procedures: 506 (87%)

Median Age: 13 m (1 day- 27 years)

Median Weight : 8.4 kg (1.9-80)

ECMO 11 runs/year (60% discharge home)

LATE PRESENTATION OF CHD

Rachs Totales % Mort%

R1 79 13.6 0,0

R2 203 36.6 0,5

R3 200 36.1 8,0

R4 52 9.3 5,8

R5/6 20 3.6 15,0

Total 554 4.2

LATE PRESENTATION OF CHD

• The opportunity for CHD repair depends on the pathology and tolerance of the patient to the disease

• The aim of early surgery repair is to avoid the negative impact of abnormal physiology on vital organs such as the CNS, lungs and the heart itself

• Early diagnosis is essential to achieve the successful treatment of CHD

• The delay deteriorates the patient´s clinical condition and increasing morbidity and mortality

LATE PRESENTATION OF CHD

• The care process, in most cases, begins when the neonatologist or pediatrician suspects CHD and less frequently its is detected by prenatal Ultra Sound (US)

• In our CICU, bet 2007 and 2011, 299 neonates were admitted 1 ü 11% without Prenatal Care ü Prenatal US:

• Health insurance: 12.5% vs. 5.1%, p= 0.047 • Living in Buenos Aires City: 8.7% versus 1.2% p= 0.019

• Prenatal US reports: 20- 50% 2,3,4

ü Brown K. (Heart 2006): 20% ü Schultz AH: (Pediatrics 2008): 44% ü McBrien A: (US Obs Gy 2010): pre training 28%- post 43%

1. Krynski M, Montonati M, et al. Impact of the time of diagnosis on the postoperative outcome of newborn infants with congenital heart disease in a public hospital in Argentina. Arch Argent Pediatr 2015;113(5):433-442.

2. Brown KL, et al. Delayed diagnosis of congenital heart disease worsens preoperative condition and out come of surgery in neonates. Heart 2006;92(9):1298-302.

3. McBrien A et al. Impact of a regional training program in fetal echocardiography for sonographers on the antenatal detection of major congenital heart disease. Ultrasound Obstet Gynecol. 2010;36(3):279-84.

4. Schultz AH et al. Epidemiologic features of the presentation of critical congenital heart disease: implications for screening. Pediatrics. 2008 , 121(4):751-7.

LATE PRESENTATION OF CHD

• The treatment of CHD is a process in which stages can be recognized:

ü Detection of the malformation

ü Adequate stabilization

ü Transfer to a more complex care center

ü Surgical repair and perioperative care

ü The treatment of CHD should end with the follow-up of the operated patients in order to evaluate results and detect further complications

LATE PRESENTATION OF CHD

• Reasons:

ü Lack of identification of the pathology

ü Difficulties in initial stabilization

ü Delays in transport to the tertiary center

ü Transport problems: unplanned ET extubation, etc

ü Surgical delays in the referral center: that particularly affect patients with out social security

Case 1

• A 1 year old girl, 8 kg • Severe cyanosis was detected in Misiones province • She was transferred to our intitution

TGA

• Echo: TGA simple with large ASD

ü What can we offer to our patient?

ü Should be operated? Comfort care?

ü Is She a good candidate for a surgery?

ü Can She tolerate surgery? ECMO?

ü Will the patient be clinically better after surgery?

TGA: LV train before Switch Operation

Between 1993- 2016, arterial switch operation were performed in 205 patients with diagnosis of simple D-TGA LV required re-trained in 13 patients *

ü Median aged: 13,1 m (2 month- 5 years) ü Rashking septostomy: 7 patients ü Surgical procedure: PAB + BTS (8 L-BTS, 5 R-BTS)

• Evolution: ü MV days after PAB: median 15,36 (4- 57) ü PAB media: 29.7 days (1 patient remained with PAB during 5 years)

* Dr. García Delucis Pablo. Data presented at the meeting of the Argentine Society of Cardiovascular Surgeons 2016

TGA: LV train before Switch Operation

• LV re-training indications:

ü Age > 2 month old

ü Poor LV Function

ü LV pressure < = to 50% of systemic Ventricle

ü LV mass < than 35 g / m2

• Betwen 21 days old and 2 month old

ü The LV is evaluated and we offer ASO with ECMO

TGA: LV train before Switch Operation

Post Banding • Complications

ü LCO

ü Poor LV function

ü Pneumothorax (7.6%)

ü Pericardial effusion (7.6%)

ü Pleural effusion (7.6%)

ü BTS thrombosis (15.8%)

ü IVC thrombosis (7.6%)

• Mortality: 2 patients pre- switch op

TGA: LV train before Switch Operation

• ASO is indicated when: ü LV function improved

ü PAB gradient 70 mmHG

ü LV mass > 50 g / m2

• ASO was sucesfully performed in 11 patients ü MV days after ASO: median 7 (3- 15)

Case 2

• A 6 month old boy, 3.5 kg • Birth weight 3.2 kg • His mother consulted because respiratory distress • He required to be intubated and was transferred to our

institution

Shunts Lesions: VSD

The admission of children with a VSD and HF suffering severe

respiratory distress waiting for the surgery:

• Preoperative stabilization with MV or NIV • It´s difficult to wean MV before the surgery • Inotropic support with dobu or dopa • Under nourished should not delay the surgery • Long LOS • PA banding VS Repair procedure?. We always consider repair surgery • After they are operated they improve their clinical condition quickly

In case of pre Op infection with SR Virus with MV support: • Difficult control the HF before surgery • We usually wait a week under MV until viral inflammatory response

decreases, before to proceed the surgery

A CC study comparing two pre Op MV groups after VSD closure*, • Morbidity was high in both groups, with ⇑ MV days and ⇑ LOS (n/s) • Mortality, 1 patient in Group II (w/o SRV infection)

(*) Congenital Heart Surgery in infants with recent respiratory virus infections. The 4º World Congress of Pediatric or Pediatric Cardiology and Cardiac Surgery. Abstract 684. 2005

Shunts Lesions: VSD

Group Age Weight (kg) Pos Op MV days LOS days

VSD/ MV & SRV infection (n=6)

7 m (2.5 m- 2 y)

5.3 (3.8- 8)

6 (2- 23)

16 (5- 34)

VSD/ MV (n=10)

4.5 m (2 m- 2.4 y)

3.4 (2.8- 8.6)

7 (2- 15)

15 (2- 28)

P value 0.73 0.15 0.69 0.52

Case 3

• A 2 years old boy, 12 kg • Down Sindrome • His mother says: ü During the first year of life he was admitted in the local hospital

because many episodes of respiratory distress ü Last semester, he improved significantly and he gain weight ü The last control he presented saturation 85% and was sent to our

institution to be evaluated

Shunts Lesions: Older CAVC

• The impact of closure of a defect in the presence of PAH with increased PVR is unknown

• When PAH is reversible?. There is no data and it is controversial • In general: PVRI PVR Surgery

< 4 UW-m2 < 2.3 Yes

> 8 UW-m2 > 4.6 No

4- 8 UW-m2 2.3- 4.6 Individualized

• In pos Op: ü Fenestrated ASD, VSD

ü Pulmonary Artery Line

ü NO, Iloprost, Sildenafil

Case 4

• A 4 years old boy, 14 kg • Squatting on the floor waiting for the first appointment • Severe Cyanosis • Finger clubbing

FALLOT

• After repair surgery • RV hypertrophy ➡ LCO

ü Severe diastolic dysfunction § Milrinone: max doses tolerated with out hypotension § CVP: > 12

ü JET: Poorly tolerated ü Pleural effusions

§ early drainage to improve pulmonary function ü Early Peritoneal dialysis

§ To evacuate ascites and decrease high intra-abdominal pressure and § To minimize fluid overload due to expansions

FALLOT

• Collaterals ü Must be pre Op ruled out and occluded before surgery

ü Decrease venous drainage in CPB

ü In case of Post op Pulmonary hemorrhage:

§ Requiring high positive pressure ventilator

§ Require urgent cath embolization

§ Differential diagnosis: residual VSD or additional undiagnosed VSD

CONSIDERATIONS

• There are very few reports in children with Late Presentation of CHD in the major journals,

• but we think there is a chance of performing a surgical treatment although the results may not be the expected ones

• Sometimes we are guided by experience, background and common sense

• This sub group of patients is not included in adjusted risk models (RACHS-1)

• They need to be categorized with more accurate risk adjustment tools

CONSIDERATIONS

• The challenge:

1. Is the standard surgical procedure appropriate for this patient?

Sometimes the option is not de typical procedure Each patient needs to be individualized

2.Will the patient withstand the surgery?

Often these patients have to be operated in poor critical conditions, with long MV days previous to surgery, bad nourish, the risk of infections is increased

3. The family

The family is informed about the risks and chances Long LOS affects family, with others children who stay in distant province. Doing nothing is mortality. And one opens a possibility

CONSIDERATIONS

• After initial stabilization (invasive monitoring, MV, inotropes) and once infections is ruled out,

• We offer to the parents the possibility of surgical procedure

With the conviction that after the repaired surgery, the patient

will be clinically better

• These children have less chance of survival, and their treatment is more expensive, requiring much more effort from the team

• We have no data of how many children in our country do not reach the surgery and die as a consequence of their CHD

• Pre Op Mortality might even be higher than the one Pos Op

Thank you!