Introduction to adult congenital heart disease

part 1

Presented byDr: Ibrahim Emam

Fellow Of Egyptian Board Of Cardiac Surgery

Introduction

•The successful management and correction of congenital heart disease( CHD) has resulted in an increase number of adult with CHD 85% of children born with CHD now survive to adulthood •40% of the operations was the first time complete and 52% were reoperation (1)

Classification

• First group: Include who undergone complete repair

• Second group: Include who undergone palliative procedure

• Third group: Include those without previous surgery (2)

Special consideration

Chronic cyanosis

Resternotomy Iron deficiency

hemoptysisstroke

ErythrocytosisHaemostaticDysfunction

CollateralArteries

Pressure and/or Volume over load

Renal dysfunction

Chronic cyanosis

1- ErythrocytosisThe red cell mass may be up to 3 times

normal→ hyperviscosity Symptoms of hyperviscosity:- Headache - Faintness - Visual disturbance- Myalgia - Muscle weakness- Paresthesias - Impaired mentation If the patients had symptoms → phlebotomy

with fluid replacement should be performed to reduce red cell mass→ ↑blood flow (3)

Chronic cyanosis …..continue

2- Haemostatic dysfunction• Thrombosis due to hyperviscosity• Bleeding due to platelet dysfunction,

thrombocytopenia, hypofibrinogenemia, accelerated fibrinolysis and clotting factor deficiencies

In patients with HB > 20 g/dl and complication of bleeding, phlebotomy of 1to2 units my improve hemostasis (4)

Chronic cyanosis ……. continue

3- Collateral arteriesTypes: communicating and uncommunicaing restrictive and unrestrictiveComplication: injury during sternotomy,

washing out of cardioplegia, volume overload

Management: - unifocalization for restrictive - catheter coil occlusion or surgical ligation for unrestrictive (5)

Chronic cyanosis …….continue

4- Renal dysfunctionDue to ↓GFR and proteinuria with segmental

sclerosis and basement membrane thickening Patients requiring cardiac cath. Should be well

hydrated before and after, minimal amount of contrast and monitoring of urine output (6)

stroke

• Children < 4 years old with iron deficiency are at increase risk of intracranial venous and sinus thrombosis

• Stroke caused by cerebral arterial thrombosis due to hyperviscosity was not established (7)

Resternotomy

• Can be hazardousAs ♥ dilated RA, RV, or RV to pulmonary

artery conduit adherent to posterior sternum

♥ If RA OR RV entered in the presence of intra-cardiac defect→ catastrophic air embolism

♥ Chronic hepatic congestion and coagulopathy will exacerbates the bleeding (8)

Resternotomy …..Continue

• So This patients need♥ Review operative report ♥ PA and lateral CXR ♥ C T scan♥ Prepare groin♥ Cannulation of femoral vessel ♥ Antifibrinolytic agents ♥ Oscillating saw ♥ Autologous blood donation ♥ Cell – saving devices♥ External defibrillator ♥ Blood be available in

OR♥ Deep hypothermia, fiberlate the heart, and keep the heart

full if re-entry is suspected (8)

hemoptysis

Causes ♥ Pulmonary infarction due to embolism ♥ Rupture enlarged friable bronchial artery Management ♥ Prompt bronchoscope to clear airway and

identify the bleeder ♥ If the bleeding persists →angio

→embolization ♥ If persists → emergent pulmonary resection

(5)

Iron deficiency

• From red blood cells lyses due to turbulence of the flow through stenotic valve or conduits and regurgitate valve

• Result in microcytic hypochromic erythrocyte →↓red blood cells compliance → ↑viscosity

• Need iron replacement with close monitoring to avoid excessive erythrocyte production and avoid dehydration (9)

Pressure and/or volume overload

• Volume overload from intra and extra- cardiac shunting → heart failure and arrhythmia

• Pressure overload from stenotic lesions→ hypertrophy → ↓compliance and arrhythmia

Surgical consideration in patients with previous

operation

Blalock - Taussing shuntIs a surgical procedure to give palliation to cyanotic heart defects which are common causes of blue baby syndrome. In modern surgery, this procedure is temporarily used to direct blood flow to the lungs and relieve cyanosis while the infant is waiting for corrective or palliative surgery( PA, F4, and tricuspid atresia). Classic: S C artery ibsialateral to the aortic arch to branch PA end to siteModified: Interposition graft( PTFE) between SC or innominate artery and the pulmonary artery

Blalock - Taussing shunt …..continue

ABP monitoring in the opposite handAvoid ↓BP during induction of anesthesia to avoid pulmonary hypoperfusion Avoid injury of the shunt during dissectionAt the start of CPB the shunt should be ligated and dividedAspirin should be discontinue 5 days before reoperation Arterioplasty for distorted pulmonary artery

Glenn shunt

The Glenn procedure is the surgery used to prepare the heart for a Fontan procedure for patients with a single ventricle.

Classic: is an end to end anatomies between right PA and SVC

Modified bidirectional: is an end to side anatomies of SVC and superior margin of right PA

The classic Glenn is take down, and PA continuity is reestablished (10)

Classic Glenn

Pulmonary artery band( PAB)

• ↓excessive pulmonary blood flow , thereby reducing the incidence of PH

• At the time of re-operation repair of stenosis must be addressed by pericardium or prosthetic patch

• If the band migrate → bilateral pulmonary artery stenosis or distortion, my necessitated homograft, prosthetic material or pericardium (11)

Pulmonary artery band

Indication of PAB

• Multiple VSD• Tricuspid atresia type IIC• TGA presented late, atrial switch

for conversion to arterial switch (11)

Anesthesia consideration

• Prevent anxiety, tachycardia, and increased myocardial demands

• Avoid myocardial depression, vasodilatation, hypotension, large tidal volumes and height PEEP especially in patients depend upon preload like Fontan patients

• Hypoxemia and acidosis are to be avoided to prevent PVC

• Minimal O2, hypercapnia and mild acidosis to prevent excessive pulmonary blood flow (12)

References1. Gatzoulis MA, Hechter S, Siu SC, et al: Outpatients clinics for

adults with congenital heart disease: increasing workload and evolving patterns of referral, Heart 81:57, 1999.

2. Moodie D: Diagnosis and management of congenital heart disease in the adult. Cardiol Rev 2001; 9:276.

3. Rosove MH, Perloff JK, Hocking WG, et al: Chronic hypoxaemia and decompensated erythrocytosis in cyanotic heart disease, Lancet 2:313, 1986.

4. Henriksson P, Varendh G Lundstrom NR: Haemostatic defects in cyanotic congenital heart disease, Br Heart J 41: 23, 1973.

5. Yasuyuki Okuda, Kan Takeda, Tomoyasu Tagami, Kaname Matsumura, Maki Ooi, and Tsuyoshi Nakagawa

– Effect of Embolization of Major Aortopulmonary Collateral Arteries on Cardiopulmonary Circulation in Congenital Heart Disease: Assessment with Lung Perfusion Scan Vasc Endovascular Surg 1995 29: 199-207.

6. Spear GS: The glomerular lesion of cyanotic congenital heart disease, Johns Hospital Med J 140:185, 1977.

References

7. Phomphutkul C, Rosenthal A, Nadas AS, et al: Cerebrovascular accidents in infants and children with cyanotic congenital heart disease, Am J Cardiol 32:329, 1973.

8. Berdat PA, Immer F, Pfammatter JP, et al: Reoperations in adults with congenital heart disease: analysis of early outcome. Int J Cardiol 2004; 93:239.

9. Rosenthal A, Nathan DG, Marty AT, et al: Acute haemodynamic effects of of red cell volume reduction in polycythemia of cyanotic congenital heart disease, Circulation 42:297, 1970.

10. Elizari A, Somerville J: Experience with the Glenn anastomosis in the adult with cyanotic congenital heart disease. Cardiol Young 1999; 9:257.

11. Oldham HN Jr, Kakos GS, Jarmakani MM, Sabiston DC Jr. Pulmonary artery banding in infants with complex congenital heart defects. Ann Thorac Surg. Apr 1972;13(4):342-50.

12. Baum,V.C., The adult patient with congenital heart disease, Journal of Cardiothoracic and vascular Anesthesia ,10(1996) 216- 82.

Nothing impossible