Cyanotic Congenital Heart Disease

Dr. Ahmad Rustam bin Mohd ZainudinMD, MMed (UKM)Paediatric CardiologistHospital Pulau Pinang

Lecture Outline

• Overview and Causes of Cyanosis

• Cyanotic Congenital Heart Disease

– Types

– Clinical Features

– Management

Cyanosis

• "Blue discolouration of the skin & mucous membrane"

• Deoxygenated Hb reaches 4 to 6 gm/dl, cyanosis may be seen.

• Normally approximately 80-87% oxygen saturation would give rise to clinically apparent cyanosis.

• However, in cases of anemia where haemoglobin levels are low, lower oxygen saturation may show up as cyanosis.

• Hb level of 6 g/dl for example shows cyanosis only when oxygen saturation has dropped below 60%

Hyperoxia test

• 100% O2 via HBO2 for 15 minutes• ABG from right radial artery• Cyanotic CHD:

– pO2 <100mmHg; or– rise in pO2 <20 mmHg

• Shortfalls:– Massive intrapulmonary shunts (eg: PPHN, AVM):

pO2 <100 mmHg– Cyanotic CHD with large pulmonary blood flow (eg:

TAPVD): pO2 may significantly rise with O2

Heart Disease in Children

Acquired Congenital

Acyanotic Cyanotic

Differential Diagnosis of CCHD

CXR

Increased Pulmonary Vascularity

Decreased Pulmonary Vascularity

ECGECG

RVH: TGA, TAPVDLVH or BVH: Truncus, Single ventricle, TGA & VSD

RVH: TOFLVH: Pulmonary Atresia, Tricuspid AtresiaBVH: Single ventricle & PS, TGA & PSRBBB: Ebstein anomaly

Differential Diagnosis of CCHD

• Increased Pulmonary VascularityTGATAPVDTruncus ArteriosusSingle Ventricle

• Decreased Pulmonary VascularityPulmonary AtresiaTricuspid AtresiaSingle ventricle with

PSTGA with PSEbstein anomaly

Most common cyanotic congenital heart disease

5Ts

TGA

TOF

TAPVD

Tricuspid Atresia

Truncus

Tetralogy of Fallot

Tetralogy of Fallot

• 5-10% of CHD• Criteria:

– Large VSD– Overriding of Aorta– Pulmonary stenosis– Right Ventricular Hypertrophy

• Clinical features:– Cyanosis, Murmur – Hypercyanotic spells

• Investigations:– CXR: "boot-shaped" heart– ECG: RAD, RVH– Echo: confirmed diagnosis

Hypercyanotic spell

• Also known as ‘tet’ spell (commonly seen in Tetralogy of Fallot)

• Characterized by:– Period of uncontrollable cry/

panic– Rapid & deep breathing

(hyperpnea)– Deepening of cyanosis– Decreased intensity of heart

murmur– Limpness, convulsions &

rarely, death• Peak age: 2-24 months

– Taksande et al, J MGIMS, 2009

How does it happen?

• Imbalance between pulmonary & systemic vascular resistance decreased pulmonary blood flow & increased right-to-left shunting.

• Results in fall of arterial PaO2• Fall in pHstimulate respiratory centre hyperpnea• Presence of fixed resistance at the RVOT more

shunting vicious cycle of hypoxic spell

– Taksande, J MGIMS, 2009

Medical Emergency!!!

• Knee-chest/ squatting position

• O2 100%• IV/IM/SC Morphine 0.1-0.2

mg/kg• IV Propanolol 0.05-0.1 mg/kg

slow bolus over 10 min (alternative: IV Esmolol/ IV Metoprolol)

• IV NaHCO3 1mEq/kg• Vasopressor +/- Inotropes• Fluid challenge• Heavy sedation, intubation &

ventilation

Management of TOF

Medical Palliative

Oral propanolol procedure/ surgery

0.2-1.0 mg/kg bd/tds RVOT stenting

BT shunt

Corrective SurgeryVSD closure & RVOT resection

(ideally at 6-12 months old)

Palliative procedures

• RVOT stenting • Modified BT shunt

Thomas-Blalock-Taussig Shunt

Helen TaussigAlfred BlalockVivien Thomas

Transposition of Great Arteries

Transposition of Great Arteries

• dTGA: 5-7% of CHD; Boys 3:1• Most common cause admission for cyanotic CHD during 1st

2 weeks of life• Criteria:

– Aorta arises anteriorly from RV– PA arises posteriorly from LV

• Other associated defect:– 50% no other defect (other than small PFO/PDA)– 30-40% VSD, 5% PS, 10% VSD with PS

• Clinical features:– Cyanosis (usually noted at birth)– Heart failure

• Investigations:– CXR: Cardiomegaly, "egg on side"– Echo: confirmed diagnosis

Management

• Simple TGA (intact ventricular septum)– Prostaglandin E infusion 5-60ng/kg/min– Balloon Atrial Septostomy (BAS)– Atrial Switch Operation (ASO) at 2-4 weeks

• TGA with VSD– ASO & VSD closure at 1-3 months

• TGA with VSD & PS– BT shunt during infancy– Rastelli repair at 4-6 years

Total Anomalous of Pulmonary Venous Drainage

Types of TAPVD

Total Anomalous of Pulmonary Venous Drainage

• 1% CHD, Boy 4:1 (infracradiac type)• Types:

– Supracardiac (50%)– Intracardiac (20%)– Infracardiac (20%)– Mixed (10%)

• Clinical features:– Obstructed: Cyanosis, respiratory distress, profound

desaturation, acidosis, pulmonary HPT, FTT– Unobstructed: Cyanosis, HF

• Investigations:– CXR: "snowman" sign– Echo: Large RA,RV. Small LA, PHT, abnormal pulmonary

venous return

Management

• Digitalis & diuretics- control HF

• Correction of metabolic acidosis

• Ventilatory support (high PEEP)

• Corrective surgery:– Obstructed: Urgent (ASAP)– Unobstructed: 4-6 months old

Tricuspid Atresia

Tricuspid Atresia

• 1-3% CHD• Criteria:

– Absent tricuspid valve, hypoplastic RV– most common (50%): normally related great arteries, small VSD,

PS• Associated defect:

– ASD, VSD, PDA (necessary for survival)– 30% TGA, COA, IAA, subaortic stenosis

• Clinical features:– Cyanosis at birth– Hepatomegaly

• Investigations:– CXR: reduced pulmonary vascularity– Echo: TA, relation of great arteries, pulmonary arteries assessment

Management

Management of Tricuspid Atresia

PG infusion +/- BAS

Restrictive PBF Unrestrictive PBF

PDA stenting/ PA banding BT shunt

Glenn shunt

Fontan (TCPC)

Truncus Arteriosus

Truncus arteriosus

• 1% CHD• Criteria:

– single arterial trunk with a truncal valve leaves the heart & gives rise to the pulmonary, systemic & coronary circulations.

• Types:– Type 1: MPA present– Type 2: No MPA– Type 3: RPA & LPA distant– Type 4: RPA & LPA from descending aorta

• Clinical features:– Cyanosis, HF

• Investigations:– CXR: cardiomegaly, increased pulmonary vascularity– Echo: confirmed diagnosis

Truncus arteriosus

Truncus arteriosus repair

Thank You