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Pediatric Diseases: Congenital Cardiac Disorder

Children’s Cardiac (Heart) Disease

They say the person who has borne with abnormalities of the heart is one in one hundred. Some are really in light status so that it is cured in naturally, some are subject to have medical surgery several times, some are limited in physical exercise not to apply any burdens on heart, and some are unable to receive complete medical treatment. There are variety people.

Cardiac disease is developed after the birth. There is a person who takes medicine all one’s life, person in mild case so that the person only limits physical exercise, or person in severe stage so that he/she needs to have medical surgery or to carry Pace Maker equipment which helps heart function. There is a person in serious stage so that the cardiac transplant is the only treatment.

Person with cardiac disease looks the same as ordinary people when no symptom appears. However, they impose restrictions on themselves for physical exercise and daily life for the symptom not to appear and control the disease and strive in the society. Especially for the children who have cardiac disease, as they are unable to play or go to school like other children, they live with having an emotional pain.

Children’s cardiac disease is grouped into three types.


Hereafter, primary disease names and treatments are explained about children’s cardiac diseases.

First, normal heart structure and functions are explained. Heart is constructed with four rooms. Each room has a valve for blood not to adversely flow. Dusky-red blood with less oxygen returning from entire body have first collected into right atrial and goes into right ventricle via large tricuspid valve. Right atrial boldly shrinks and discharge blood to Lung.

Heart structure and function. l blood turns into clear red color after absorbing much oxygen from lung once gathered at left atrial then goes into left ventricle through large mitral valve. Left ventricle boldly shrink and discharge artery blood out in every corner of entire body. Oxygen is released out at every muscles or organs in the body, carbon dioxide collected blood, called as venous blood, return to heart again.

Congenital Cardiac disorder appears at one in 100 children.

As explained before, approximately one in 100 baby has some kinds of disease in nature. Disease in which heart abnormality exists in nature is called as Congenital Cardiac Disease.

It is said that several factors are complexed and affected as the cause of the disease, so that it is hard to specify in most of the cases. Genetic contributor might be the cause of this disease, but rather, it is said that more than 90 percent, disease is the result of several environmental factors are complexed. Thus, the cause of the disease is unknown.

The provability ratio, one in 100, remains unchanged in past 30 years. Circumstances of life or social aspect changes has no relation with the disease. It is therefore considered that the minor glitch occurred in the birth process cause abnormalities on organ growth and formation.

However, these figures, one in 100, means the number of babies who have borne with energy. They have had a zest for living. (i.e. There are babies who dies at birth.)

Interventricular septal defect takes 60 percent of congenital cardiac disorder.

Congenital Cardiac Disorder can be divided into; (1) the case that mass blood goes in circles between heart and lung through the hole on heart, so that heart or lung bears a heavy burden. (acyanotic cardiac disease), (2) the case that because of dusky-red venous blood with less oxygen discharged out from main artery to entire body through the hole on heart, lips or hands-feet turns into purple color (Cyanotic cardiac disease).

In the Acyanotic cardiac disease, symptoms of cardiac disorder, such as breathing out fast painfully, sweating much, unable to drink milk too much, and weight do not increase, are appeared. When baby has caught a cold, breathing status might be getting worse.

In Cyanotic cardiac disease, weight increase itself is relatively good, but it may turn into serious status when baby start crying, pushing, or developing a fever so that entire body turns into purple color.

Congenital Cardiac Disorder has a variety of clinical state like, really mild case which no treatment is required, natural healing case, case for which urgent surgery is required, or case which is severe or intractable.

Table: Repetition by type of Congenital Cardiac Disorder
Name of disease Numbers of cases
Interventricular Septal Defect (VSD) 433 56.0
VSD + another left-right shunt 31 4.0
Pulmonary Artery Stenosis 74 9.6
Atrial Septal Defect 41 5.3
Tetralogy of Fallot 35 4.5
VSD + PS 6 0.8
Patent Ductus Arteriosus 28 3.6
Aortic Coarctation/release 21 2.7
Complete transposition of great arteries 17 2.2
Endocardial Cushion Defect 14 1.8
Double-Outlet Right Ventricle 10 1.3
Total Anomalous Pulmonary 9 1.2
Spleen Formation Incomplete Disease 7 0.9
Right Ventricular Hypoplasia Syndrome 6 0.8
Single Ventricle Disease 5 0.6
Left Ventricular Hypoplasia Syndrome 5 0.6
Tricuspid Atresia, Epstein malformation, Total Artery Trunk Remnant, Aortic Coarctation, Corrected Transposition of Great Artery, Peripheral PS 各3 各0.4
Mitral Incompetence 2 0.3
Cardiac Myopathy, cor triatriatum 1 0.1
Mitral Valve Stenosis / Closure Insufficiency Disease 1 0.1
Unknown Name Disease 8 1.0
* Spleen Formation Incomplete Disease=Alienia or Polysplenia, Right Ventricular Hypoplasia Syndrome=Pure Pulmonary Atresia

Makoto Nakazawa, et al: Development Status of Baby Cardiac Disease in Japan Japan Society of Pediatric Cardiology and Cardiac Surgery magazine 29:2597, issued in 1986

The most common disease among congenital cardiac disease in Japan is the Atrial Septal Defect. Above tables shows the data supplied by Tokyo Women’s Medical University, it takes approximately 60 percent of congenital cardiac disorder. Atrial septal defect is a disease having a hole on the wall in the heart separating left ventricle and right ventricle. If this hole is small enough, it may be cured naturally at one out of 5, but in case it is large one, medical surgery is performed to close this hole to prevent backflow of blood.

The next highest number is Pulmonary Artery Stenosis (about 10%). Pulmonary Artery is a vein connecting heart and lung, but because of it becomes narrow (stenosis), blood becomes hardly flow from heart to lung. In case is mild one, no treatment is necessary but in case stenosis becomes middle to severe

he third highest number is Atrial Septal Defect (about 5%). Atrial Septal Defect is a disease where a hole is developed on the wall separating right atrial and left atrial. A hole which would not be cured naturally, is removed by medical surgery, but recently treatment becomes subject to Health Insurance so that catheter caulking is getting popular. However, there is a location where catheter is unable to caulk out.

Tetralogy of Fallot is the disease where four disease, Atrial Septal Defect, Pulmonary Artery Stenosis, Aortic Right Dislocation, and Right Ventricle Hypertrophy, has combined so that cyanosis is developed. This is the highest disease among the cyanosis developing diseases, but for most of them the medical surgery treatment is possible.

Other than the above, there are diseases, such as Patent Ductus Arteriosus which arterial duct remains open which should close at birth, complete transposition of great arteries which main artery and artery of the lung are reversely attached, single ventricle disease which cardiac has only one chamber, or left ventricular hypoplasia syndrome which has insufficient formation of left ventricle.

Valvular disease caused by stenosis of the valve or closure insufficiency.

There are valves in the heart to control and adjust blood flow. Tricuspid valve between right atrial and right ventricle, pulmonary valve between right ventricle and pulmonary artery, mitral valve between left atrial and left ventricle, and aortic valve between left ventricle and main artery.

When these valves do not open properly (arctation) or not close properly (closure insufficiency), heart receives much stress then turns into cardiac arrest.

There is a person who has abnormality on these valves by nature. For the mild case, treatment is unnecessary till growing up, or throughout entire life period. For severe case, like person who has palpitation or strong cardiac murmur, surgery for these valves are necessary.

Mitral valve stenosis and closure insufficiency disease are the common disease among Japanese. Plastic surgery which pull mitral valve into shape, or when unable to form, replacement surgery which replace valve with artificial valve.

Heart activity beats when young so that if replaced with artificial valve, it become calcified in early stage or blood clot is developed. If that is a case, repeat surgery is necessary. The risk of Cardiac surgery becomes high for repeat surgery so that there is a case which first surgery timing is postponed as much as possible. For that reason, medical therapy or exercise limitation may conduct.

Discovery rate for baby in the womb is 70 to 90 percent.

Recently, congenital cardiac disorder can be found when baby in the womb by echography (echo). At earliest, at 20 weeks (5 months) pregnancy when heart can be seen in small, cardiac disorder is found out. Mostly, when the heart of baby in the womb grow in 7 to 8 months pregnancy, it’s size and structure are diagnosed by Echo. It is hard to say everything can be found by this (70 to 90% can be found), but if heart disorder is found when they are baby in the womb, postnatal countermeasures can be planned prenatally thus it becomes able to perform adequate treatment at early stage, so that it becomes possible to treat or save the disease in greater degree of severity.

Also, there is a case that atrial septal defect, which no symptom appears when young, is found when heart is getting larger as grown or heart activity has increased with physical exercise.

There are many people who are found atrial septal defect, as first time, at the school physical examination. Or, symptom has just appeared at the age of 60s and 70s, or person lives out their natural life without even noticing.

To grasp diagnosis and disease condition, chest X-Ray, cardiac electrogram, electrocardiographic, cardiac catheter test, CT/MRI examination, and/or blood test may be conducted.

Cardiac Surgery for Child

Currently, the risk rate of cardiac surgery to the child is 3 to 4 % in overall. If the symptom is severe, it is 30%, if mild, case fatality rate is less than 1%. World’s first cardiac surgery was made in 1950s, it is roughly 50 years history. There is no tools or medicine at that time but better surgical equipment and cardiac stimulant are developed, and surgical technic has also improved so that success rate jumped up dramatically. Especially, for severe congenital cardiac disease, which has only one sufficiently functioning cardiac chamber, like common ventricle, pulmonary valve closure, tricuspid atresia, and left ventricular hypoplasia, Fontan surgery is widely spread out thus the life-saving rate has increased greatly as the result.

Many can be saved life, if disease is found in early stage and apply appropriate medical treatment at appropriate timing. In case severe and complicated cardiac disease, phased surgery is necessary. Also, it is difficult to acquire 100% QOL and there is a case where to apply limitation on daily life.

The risk rate is getting higher on the repeated cardiac surgery. Once take a scalpel to the body, the cut is coalesced so that the surgery is getting difficult due to much bleeding. Therefore, for person who has received cardiac surgery few times, cardiac surgery may not become applicable.

Nowadays, it is expected that the objectives of the surgery will be improved further towards QOL maintained treatment through entire life, from simply life-saving treatment.