"Nature is neither lazy nor devoid of foresight.

Having given the matter thought, she knows in advance that the lung of the fetus does not require the same arrangements of a perfected lung. She/he has therefore anastomosed the pulmonary artery with the aorta, and the left and right atria. . .. -Galen, 2nd Century

Foetal circulation & congenital heart disease

Ms.Dhanalakshmy First year MSc nursing.

 Fetal circulation can be defined as the system of blood vessels and structures through which blood moves in a fetus from the placenta

TEMPORARY STRUCTURES IN FOETAL CIRCULATION.

Flow Chart of Fetal Circulation

Umbilical blood flow rate is 180ml/kg of fetal weight.

TRANSITIONAL CIRCULATION
AT BIRTH
Mechanical expansion of lungs Increase in arterial PO2

Rapid DECREASE in pulmonary vascular resistance

Removal of the low-resistance placental circulation INCREASE in systemic vascular resistance.

Right ventricle output now flows entirely into the pulmonary circulation.

Pulmonary vascular resistance becomes lower than systemic vascular resistance. Shunt through ductus arteriosus reverses & becomes left to right.

High arterial PO2 (In several days)

Constriction of ductus arteriosus and become ligamentum arteriosum .

Increased volume of pulmonary blood flow returning to left atrium

Increases left atrial volume and pressure

Closure of foramen ovale (functionally) (Although the foramen may remain probe patent)

Becomes Fossa Ovalis

Removal of the placenta from the circulation

Becomes ligamentum venosum

Also results in closure of the ductus venosus.

Contd..

Umbilical vein

Ligamentum teres.

Contd..

Umbilical artery

Median umbilical Ligament.

CLOSRE OF :
Foramen ovale: Anatomical closure: 1 year of age. Functional closure: 3rd month of life Ductus arteriosus: Anatomical closure: Several weeks. Functional closure: 10-15 hr in a normal neonate.

FETAL Gas exchange RV,LV circuit Placenta Parallel

NEWBORN Lungs Series

Pulmonary circulation

Vaso-constriction

Vaso-dilation

Fetal myocardium
Contractility,Compliance Dominant ventricle Change in Structure

Less Right Umbilical vein Umbilical artery Ductus venosus Ductus arteriosus Foramen ovale

Good Left Ligamentum teres Medial umb ligament Ligamentum venosum Ligamentum arteriosum Fossa ovalis

congenital heart disease

Congenital Heart Disease

A general term to describe abnormalities of the heart or great vessels that are present from birth. Most arise from faulty embryogenesis during gestational weeks 3 through 8, when major cardiovascular structures develop.

Overview

CHD is the most common type of heart disease among children.

0.5-0.8% of live births.

Etiology and Pathogenesis

Unknown : > 90% of patients. However, defect in some of the cases may be due to: 1. Genetic disorder : two to tenfold increase (Downs syndrome). 2. Maternal infection: Rubella virus infection during first trimester, especially during the first four weeks of gestation. 3. Use of drugs: Example: Use of thalidomide, aspirin etc. during pregnancy.

Contd
4.Sex:female-right sided lesions. male-left sided lesions. 5.Environment: high altitude: risk of PDA&

ASD.
6.Maternal conditions: diabetic and alcoholic mothers. 7.Heredity: siblings with CHD.

Prevention
The health protection of pregnant woman should be enhanced.
High risk factors, such as drugs, radiation, viral infection, et.should be avoided. Suit dosage Folic Acid should be filled up in early pregnancy stage.

EVALUATING A CHILD WITH MURMUR

Does the child have heart disease?

Is it congenital heart disease?

If it is congenital heart disease, what is the lesion?

What is the severity of the lesion?

Assessment of a child for the presence of heart disease.

Nadas criteria.

1.

2.

3. 4.

Major Systolic murmur garde III or more specially with a thrill Diastolic murmur Cyanosis Congestive heart failure

Minor 1.Systolic murmur less than grade III in intensity. 2. Abnormal S2. 3. 3.Abnormal ECG. 4.Abnormal X-ray. 5. Abnormal BP

Congenital Heart Disease

Three major categories: 1. Malformations causing a left-to-right shunt (Acyanotic heart disease) 2. Malformations causing a right-to-left shunt (Cyanotic heart disease) 3. Malformations causing an obstruction (Stenosis / Atresia).

Congenital Heart Disease Terms:

Shunt : An abnormal communication between chambers or blood vessels. Permits the flow of blood from left to right or the reverse, depending on pressure relationships.

Contd...
Atresia : A complete obstruction. Hypoplasia : A decrease in the volume and muscle mass of a cardiac chamber which occurs before birth. Atrophy : A decrease in the volume and muscle mass of a cardiac chamber which occurs after birth.

ACYANOTIC HEART DEFECTS

Left-to-right shunt

Atrial Septal Defect (ASD)

Atrio-ventricular canal Defect

Ventricular Septal Defect (VSD)

Patent Ductus Arteriosus

CYANOTIC HEART DEFECTS

right-to-Left shunt

Tetralogy of Fallot

Ebsteins anomaly

Transposition of the Great Arteries (TGA)

Tricuspid Atresia

Total anomalous pulmonary venous connection

Obstructive Congenital Anomalies

Aortic Valve Stenosis

Coarctation of Aorta

Pulmonary Valve Stenosis

SUMMARY.
FOETAL CIRCULATION. CONGENITAL HEART DISEASE. Definition,incidence. Etiology & prevention. Evaluating a child. Nadas criteria. Classification of CHD: Left to right shunt. Right to left shunt. Obstructive anomalies.

Assignment.

References:
Ghai.o.p.ghai essential pediatrics Kleigman.nelson textbook of pediatrics Dutta.d.c.textbook of obstetrics Chaurasia.b.d.human anatomy

 Teachers open the door but you must walk through it yourself.

ACYANOTIC HEART DEFECTS

Left-to-right shunt

Defined:
A congenital disorder manifested with left to right shunting and obstructive lesions. Clinical signs are not always apparent at birth, they manifest anytime during infancy or early childhood.

Defects:
The blood is shunted through an abnormal opening from the left side of the heart to the right side of the heart. Pulmonary blood flow increases because of the extra volume in the right side.

Exposure of the postnatal, low pressure, low resistance pulmonary circulation to increased pressure and/or volume results in RVH and later, failure.
Later, increase of pulmonary vascular resistance toward systemic levels results in reversal shunting (right-to-left) Eisenmenger Syndrome.

Examples:
Atrial Septal Defect (ASD),
Ventricular Septal Defect (VSD), Patent Ductus Arteriosus (PDA), and

Atrioventricular Septal Defect (AVSD).

Atrial Septal Defect (ASD)

Incidence and Pathophysiology:

An abnormal opening between the atrial septum that allows communication of blood between the left and right atria.
Most common congenital cardiac anomaly in adults (10% of all CHDs). F>M

Atrial Septal Defect (ASD)

Atrial Septal Defect (ASD)

Types of Lesions according to their location in the septum: 1. Ostium Secundum: A defect located at and resulting from a deficient or fenestrated fossa ovalis. The most common type (90%). Usually isolated. The atrial aperture may be:
Single Multiple or
Fenestrated.

Atrial Septal Defect (ASD)

2. Ostium Primum:
5% of ASDs. Occur low in the atrial septum, adjacent to the AV valve Results from a defect in endocardial tissue formation Often associated with a cleft anterior mitral leaflet (Partial AV septal defect).

3. Sinus Venosus
5% Located high in the septum, near the entrance of the SVC. Commonly accompanied by anomalous connections of right PVs to the SVC or RA.

Atrial Septal Defect (ASD)

Altered Hemodynamics:
Left-to-right shunt results because pulmonary resistance is considerably < than systemic vascular resistance and the compliance (distensibility) of the RV is > than that of the LV. This increased blood flow (2 to 4 times the normal) through the pulmonary valve leads to an enlarge RA and RV and increased pulmonary blood flow.

Manifestations:
Most infants and children are asymptomatic but over years (after 30yrs) to decades may experience: 1. Fatigue and SOB 2. Palpitations or atrial dysrythmias result of atrial enlargement 3. Recurrent respiratory infections can occur when there is a large amount of pulmonary blood flow 4.Systolic murmur is produced by increased blood flow across the pulmonary valve. 5. Diastolic murmur is present with large shunts. 6. Stroke or major organ damage can occur because of embolization of thrombus, air or other materials PARADOXIMAL EMBOLISM. 7. HF and irreversible pulmonary disease.

Ventricular Septal Defect (VSD)

Incidence and Pathophysiology:
Most common congenital cardiac lesion (25% of all CHDs) Often accompanied by other cardiac defects. An abnormal opening in the ventricular septum that allows free communication between left and right ventricles.

Ventricular Septal Defect (VSD)

Classification according to size and location:
1. Membranous VSD : In the region of membranous septum. About 90%.
2. Infundibular VSD : Below the pulmonary valve

3. Within the muscular septum.

Often single, may be multiple (Swiss cheese)

Ventricular Septal Defect (VSD)

Altered Hemodynamics: The degree of left to right shunting through the VSD depends on the size of the defect and the pulmonary vascular resistance compared with the systemic vascular resistance. The pulmonary vascular system is high in the newborn. Over the first few weeks of life, the resistance decreases. As this occurs, an increased amount of blood shunts left to right of the VSD level. The pulmonary vascular circulation receives increased pulmonary blood flow. With large defects the pulmonary arteries are exposed to systemic pressures, causing pulmonary hypertension, and over time, progressive pulmonary vascular disease.

Ventricular Septal Defect (VSD)

Ventricular Septal Defect (VSD)

Manifestations:
Signs and symptoms vary with the size of the defect and the presence of associated cardiac lesions. Clinical symptoms are usually not seen at birth because of continued high pulmonary vascular resistance in the newborn. Infants with moderate to large defects will become symptomatic within the first few weeks of life. Children with small asymptomatic. defects will remain

Clinical Manifestations
Tachypnea, dyspnea Poor growth Palpable thrills Systolic murmur at left lower sternal border Shortness of breath Failure to gain weight Fast heart rate Pounding heart Frequent respiratory infections

Complications:
Congestive heart failure. Growth failure, especially in infancy. Bacterial endocarditis. Irregular heartbeat or rhythm. Pulmonary artery hypertension.

Patent Ductus Arteriosus

In the intrauterine life the ductus arteriosus permits blood flow between the aorta (distal to the left subclavian artery) and the pulmonary artery. In a full-term infant, the ductus usually closes within the first day or two of life. In premature infants and in infants with respiratory distress syndrome (hypoxemia), the ductus may remain patent causing a permanent structural defect in the wall PDA. 90 % : isolated defects. Remaining cases associated with VSD, COA, PS or AS. Associated with LVH and pulmonary artery dilatation.

Patent Ductus Arteriosus

Initially asymptomatic, long-standing case induces pulmonary hypertension with subsequent right ventricular hypertrophy and finally right-to-left shunt producing cyanosis. Continuous harsh murmur (machinery murmur) may be heard. PDA should be closed early in life (life threatening). Can be life saving in infants with various forms of CHD with obstructed pulmonary or systemic blood flow (Aortic valve atresia).

Atrioventricular Septal Defect (AVSD) / Complete AV Canal Defect

Results from abnormal development of the embryologic AV canal in which the superior and inferior endocardial cushions fail to fuse adequately, resulting in incomplete closure of the AV septum and inadequate formation of tricuspid and mitral valves.

Atrioventricular Septal Defect (AVSD) / Complete AV Canal Defect

Two most common types: 1. Partial: Primum ASD + cleft anterior mitral leaflet Mitral insufficiency 2. Complete: Large combined AV septal defect + a large common AV valve essentially a hole in the center.
All four chambers freely communicate. Volume hypertrophy of each. > 1/3 of patients Down syndrome Surgical repair.

CYANOTIC HEART DEFECTS

Right - Left - Shunts

Defined:
A congenital disorder manifested by right-to-left shunt resulting in diminished pulmonary blood flow and permitting unoxygenated or desaturated blood to enter the systemic circulation.

Right-to-left shunt (cyanotic heart disease) Cause cyanosis early in postnatal life.
Bland or septic emboli arising in peripheral veins can bypass the normal filtration action of the lungs, directly entering the systemic circulation : PARADOXYCAL EMBOLISM (Brain infarction and abscess) Long standing clinical findings : hypertrophic osteoarthropathy (clubbing of tips of fingers and toes) Polycythemia.

Examples:
Tetralogy of Fallot (TOF)

Transposition of great arteries (TGA) Truncus Arteriosus

Tricuspid Atresia Total Anomalous Pulmonary Venous Connection (TAPVC)

Tetralogy of Fallot
Incidence and Pathophysiology: 8%-10% of all CHDs, Most common cyanotic lesion in older children and adults. All of the following features result embryologically from anteriosuperior displacement of the infundibular septum.

Tetralogy of Fallot
Four anatomic changes of Tetralogy of Fallot are: 1. Ventricular septal defect 2. Dextroposition of the aorta overriding the VSD. 3. Subpulmonary stenosis with right ventricular outflow obstruction. 4. Right ventricular hypertrophy

Tetralogy of Fallot

Tetralogy of Fallot
The heart is often enlarged (boot shaped) owing to marked RVH. Additional cardiac anomalies may be present. Usually accompanied by clubbing of the fingers.

Tetralogy of Fallot
Severity of symptoms is directly related to the extent of right ventricle outflow obstruction. With a large VSD and mild pulmonary valvular stenosis, there is a mild left to right shunt without cyanosis : pink tetralogy.

More severe pulmonary stenosis produces a cyanotic right-to-left shunt. Cyanosis is present from birth or soon after.

Tetralogy of Fallot
With complete pulmonary obstruction, survival is permitted only by flow through a PDA or dilated bronchial arteries. Uncorrected Tetralogy of Fallot is a fatal disorder and leads to: 1. Heart failure, 2. Polycythemia, 3. Increased blood coagulability and thrombotic tendency, 4. Cerebral infarction, 5. Infective endocarditis 6. Brain abscess.

Tetralogy of Fallot

Complete surgical repair in classic TOF.

More complicated with Pulmonary atresia and dilated bronchial arteries.

Transposition of the Great Arteries (TGA)

A birth defect causing a fatal condition in which there is a reversal, or switch, in the truncal connections of the two main (great) blood vessels to the heart, the aorta and pulmonary artery. The aorta arises from the right ventricle and the pulmonary artery from the left.

Transposition of the Great Arteries

Transposition of the Great Arteries

Fetal development occurs as a result of mixing venous and systemic blood through the PDA and a patent foramen ovale. Therefore, postnatal life critically depends on continued patency of the ductus as well as VSD, ASD, or patent foramen ovale.

Transposition of the Great Arteries (TGA)

Prognosis depends on the severity of tissue hypoxia and the ability of the right ventricle to maintain aortic flow. If untreated, most children die within the first months of life. This malformation is particularly common in children of diabetic mothers, and it causes cyanosis.

Truncus Arteriosus
Developmental failure of separation of the embryologic truncus arteriosus into the aorta and pulmonary artery. Results in a single great artery that receives blood from both ventricles accompanied by an underlying VSD, and that gives rise to the systemic, pulmonary and coronary circulation. 1 4% of all CHDs.

Truncus Arteriosus

Truncus Arteriosus
Early cyanosis as a result of right-toleft shunting. Eventually, the flow reverses with development of RVH with pulmonary vascular hypertension. The anomaly carries a poor prognosis.

Tricuspid Atresia
Complete occlusion of the TV orifice.

Results embryologically from unequal division of AV canal.

Thus, MV is larger than normal. Almost always associated with hypoplasia of RV. The 3rd most common cyanotic cardiac condition (2 3% of all CHD).

Tricuspid Atresia

Tricuspid Atresia
Circulation is maintained by right-toleft shunt through an interatrial communication (ASD or patent foramen ovale). VSD can be present. Cyanosis is present virtually from the birth. High mortality rate in the 1st few weeks or months of life.

Total Anomalous Pulmonary Venous Connection (TAPVC)

Anomaly in which no Pulmonary vein join the left atrium. Results embryologically when the common pulmonary vein fails to develop or becomes atretic, causing primitive systemic venous channels from the lungs to remain patent. Usually drains into the left innominate vein or to enter the coronary sinus.

TAPVC
A patent foramen ovale or ASD allows pulmonary venous blood to enter the LA. Consequences :
Volume and pressure hypertrophy of the RA and RV. Dilation of pulmonary trunk. Hypoplastic LV. Cyanosis may be present.

Obstructive Congenital Anomalies

1. Coarctation of Aorta 2. Pulmonary Stenosis and Atresia 3. Aortic Stenosis and Atresia

Defects:
Obstructive or stenotic lesions : Stenosis of an opening can occur in a valve or vessel constricting or obstructing blood flow through the area. Pressure rises in the area behind the obstruction; Blood flow distal to the obstruction may be decreased or absent. Physiologic effects of obstructive or stenotic lesions :
Increased cardiac workload and ventricular strain, Clinical consequence of CHF, decreased CO and pump failure.

Coarctation of Aorta
Coarctation (narrowing or constriction) of the aorta occurs mostly (50 %) as isolated defects. The remainder of cases occur with multiple other anomalies. In most cases, cardiomegaly (chronic pressure overload hypertrophy) occurs. Clinical manifestations depend on the location and severity of the constriction.

Coarctation of Aorta :
Preductal Coarctation (infantile) / with a PDA Tubular hypoplasia of the aortic arch proximal to a PDA. Often asymptomatic in early childhood. Manifests early in life and may be rapidly fatal. Survival depends on the ability of the ductus arteriosus to sustain blood flow to the distal aorta and lower body adequately.

Coarctation of Aorta :
Preductal Coarctation (infantile) / with a PDA Lower body cyanosis. Head and arms are unaffected because their blood supply derives from vessels having origins proximal to the ductus. Usually involves a 1 to 5 cm segment of aortic root. Often associated with RVH and early right-sided heart failure.

Coarctation of Aorta :
Postductal Coarctation (adult) / without a PDA

A discrete ridge like infolding of the aorta, just opposite to the closed ductus arteriosus (ligamentum arteriosus) distal to the arch vessels.
Generally asymptomatic unless severe.

Coarctation of Aorta :
Postductal Coarctation (adult) / without a PDA It usually leads to upper extremity hypertension but low flow and hypotension in the lower extremities, causing arterial insufficiency (claudication, cold sensitivity). Collateral vessels enlarge in an attempt to bridge the gap between the upper and lower aortic segments.

Coarctation of Aorta :
Postductal Coarctation (adult) Prognosis: Even without treatment, mean life span is 40 years. Death is due to: i. congestive heart failure, ii. aortic dissection proximal to the coarctation, iii.intracranial hemorrhage or iv.infective endocarditis at the site of narrowing.

Aortic Valve Stenosis and Atresia:

Three major types aortic stenosis: 1. Valvular : i. Hypoplastic (small) ii. Dysplastic (thickened, nodular) iii.Abnormal in number (acommissural or unicommissural).

2. Subvalvular
3. supravalvular.

Aortic Valve Stenosis and Atresia:

Vulvular Aortic Stenosis: In severe congenital aortic stenosis or atresia, obstruction of the left ventricular outflow tract leads to hypoplasia of the LV and ascending aorta.
HYPOPLASTIC LEFT HEART SYNDROME

The ductus must be open to allow blood flow to the aorta and coronary arteries.

Hypoplastic Left Heart Syndrome

Aortic Valve Stenosis and Atresia:

Vulvular Aortic Stenosis: Nearly fatal in the 1st week of life, when the ductus closes. Less severe degree of congenital stenosis may be compatible with long survival.

Isolated lesion in 80% of cases.

Aortic Valve Stenosis and Atresia:

Subaortic Stenosis: Represents either a thickened ring (discrete type) or collar (tunnel type) of dense endocardial fibrous tissue below the level of cups.

Aortic Valve Stenosis and Atresia:

Supravulvular Aortic Stenosis: An inherited form of aortic dysplasia in which the ascending aortic wall is greatly thickened, causing luminal constriction.

Aortic Valve Stenosis and Atresia:

The fate of this anomaly: Infective endocarditis, LVH (pressure overload), Poststenotic dilatation of the aortic root. Clinically : low blood pressure and consequent fainting . Rare cases : sudden death. Aortic atresia may be associated with endocardial fibroelastosis.

Pulmonary Valve Stenosis and Atresia:

Obstruction (stenosis or atresia) at the pulmonary valve with intact interventricular septum. It may occur in isolation or with other anomalies. With complete pulmonary atresia, there is almost always a hypoplastic RV and an ASD with blood entering the lungs via a PDA.

Pulmonary Valve Stenosis and Atresia:

Pulmonary Valve Stenosis and Atresia:

Pulmonary stenosis is generally caused by the fusion of the cusps and may vary from mild to severe. Pulmonary outflow obstruction may also be subvalvular or supravalvular or even multiple. Mild stenosis is generally asymptomatic. Progressively more severe stenosis cause increasing cyanosis with earlier onset.

Summary

 Congenital Heart Disease

A general term to describe abnormalities of the heart or great vessels that are present from birth.

Congenital Heart Disease

Three major categories: 1. left-to-right shunt (Acyanotic heart disease) 2. right-to-left shunt (Cyanotic heart disease) 3. obstruction (Stenosis / Atresia).

Acyanotic Heart Disease

Atrial Septal Defect (ASD),
Ventricular Septal Defect (VSD), Patent Ductus Arteriosus (PDA), and

Atrioventricular Septal Defect (AVSD).

Cyanotic Heart Disease

Tetralogy of Fallot (TOF)

Transposition of great arteries (TGA) Truncus Arteriosus

Tricuspid Atresia Total Anomalous Pulmonary Venous Connection (TAPVC)

Obstructive Congenital Anomalies

1. Coarctation of Aorta 2. Pulmonary Stenosis and Atresia 3. Aortic Stenosis and Atresia

 Teachers open the door but you must walk through it yourself.