WATCH A VIDEO: Baby Born With External Heart (Ectopia cordis)

First Overview Of Heart

The heart is a muscular organ in most animals, which pumps blood through the blood vessels of the circulatory system.[1] Blood provides the body with oxygen and nutrients, as well as assists in the removal of metabolic wastes.[2] In humans, the heart is located between the lungs, in the middle compartment of the chest.[3]

In humans, other mammals, and birds, the heart is divided into four chambers: upper left and right atria; and lower left and right ventricles.[4][5] Commonly the right atrium and ventricle are referred together as the right heart and their left counterparts as the left heart.[6] Fish, in contrast, have two chambers, an atrium and a ventricle, while reptiles have three chambers.[5] In a healthy heart blood flows one way through the heart due to heart valves, which prevent backflow.[3] The heart is enclosed in a protective sac, the pericardium, which also contains a small amount of fluid. The wall of the heart is made up of three layers: epicardium, myocardium, and endocardium.[7]

The heart pumps blood with a rhythm determined by a group of pacemaking cells in the sinoatrial node. These generate a current that causes contraction of the heart, traveling through the atrioventricular node and along the conduction system of the heart. The heart receives blood low in oxygen from the systemic circulation, which enters the right atrium from the superior and inferior venae cavae and passes to the right ventricle. From here it is pumped into the pulmonary circulation, through the lungs where it receives oxygen and gives off carbon dioxide. Oxygenated blood then returns to the left atrium, passes through the left ventricle and is pumped out through the aorta to the systemic circulation−where the oxygen is used and metabolized to carbon dioxide.[8] The heart beats at a resting rate close to 72 beats per minute.[9] Exercise temporarily increases the rate, but lowers resting heart rate in the long term, and is good for heart health.[10]

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Cardiovascular diseases (CVD) are the most common cause of death globally as of 2008, accounting for 30% of deaths.[11][12] Of these more than three quarters are a result of coronary artery disease and stroke.[11] Risk factors include: smoking, being overweight, little exercise, high cholesterol, high blood pressure, and poorly controlled diabetes, among others.[13] Cardiovascular diseases frequently do not have symptoms or may cause chest pain or shortness of breath. Diagnosis of heart disease is often done by the taking of a medical history, listening to the heart-sounds with a stethoscope, ECG, and ultrasound.[3] Specialists who focus on diseases of the heart are called cardiologists, although many specialties of medicine may be involved in treatment

Location and shape

The human heart is situated in the middle mediastinum, at the level of thoracic vertebrae T5-T8. A double-membraned sac called the pericardium surrounds the heart and attaches to the mediastinum.[15] The back surface of the heart lies near the vertebral column, and the front surface sits behind the sternum and rib cartilages.[7] The upper part of the heart is the attachment point for several large blood vessels – the venae cavae, aorta and pulmonary trunk. The upper part of the heart is located at the level of the third costal cartilage.[7] The lower tip of the heart, the apex, lies to the left of the sternum (8 to 9 cm from the midsternal line) between the junction of the fourth and fifth ribs near their articulation with the costal cartilages.[7]

The largest part of the heart is usually slightly offset to the left side of the chest (though occasionally it may be offset to the right) and is felt to be on the left because the left heart is stronger and larger, since it pumps to all body parts. Because the heart is between the lungs, the left lung is smaller than the right lung and has a cardiac notch in its border to accommodate the heart.[7] The heart is cone-shaped, with its base positioned upwards and tapering down to the apex.[7] An adult heart has a mass of 250–350 grams (9–12 oz).[16] The heart is typically the size of a fist: 12 cm (5 in) in length, 8 cm (3.5 in) wide, and 6 cm (2.5 in) in thickness.[7] Well-trained athletes can have much larger hearts due to the effects of exercise on the heart muscle, similar to the response of skeletal muscle.[7]

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Nerve supply

The heart receives nerve signals from the vagus nerve and from nerves arising from the sympathetic trunk. These nerves act to influence, but not control, the heart rate. Sympathetic nerves also influence the force of heart contraction.[32] Signals that travel along these nerves arise from two paired cardiovascular centres in the medulla oblongata. The vagus nerve of the parasympathetic nervous system acts to decrease the heart rate, and nerves from the sympathetic trunk act to increase the heart rate.[7] These nerves form a network of nerves that lies over the heart called the cardiac plexus.[7][31]

The vagus nerve is a long, wandering nerve that emerges from the brainstem and provides parasympathetic stimulation to a large number of organs in the thorax and abdomen, including the heart.[33] The nerves from the sympathetic trunk emerge through the T1-T4 thoracic ganglia and travel to both the sinoatrial and atrioventricular nodes, as well as to the atria and ventricles. The ventricles are more richly innervated by sympathetic fibers than parasympathetic fibers. Sympathetic stimulation causes the release of the neurotransmitter norepinephrine (also known as noradrenaline) at the neuromuscular junction of the cardiac nerves. This shortens the repolarization period, thus speeding the rate of depolarization and contraction, which results in an increased heart rate. It opens chemical or ligand-gated sodium and calcium ion channels, allowing an influx of positively charged ions.[7] Norepinephrine binds to the beta–1 receptor.

WATCH A VIDEO: Baby Born With External Heart (Ectopia cordis)

Ectopia cordis (Greek: “away / out of place” + Latin: “heart”) is a congenital malformation in which the heart is abnormally located either partially or totally outside of the thorax. The ectopic heart can be found along a spectrum of anatomical locations, including the neck, chest, or abdomen. In most cases, the heart protrudes outside the chest through a split sternum.

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Pathology

Ectopia cordis results from a failure of proper maturation of midline mesoderm and ventral body wall formation during embryonic development. The exact etiology remains unknown, but abnormalities in the lateral body wall folds are believed to be involved. Normally, the lateral body walls are responsible for fusion at the midline to form the ventral wall. Corruption of this process may underlie ectopia cordis.

Defective ventral body wall formation yields a heart unprotected by the pericardium, sternum, or skin. Other organs may also have formed outside the skin, as well. Many cases of ectopia cordis have associated congenital heart defects, in which the heart has failed to properly form.

Defects more commonly associated with ectopia cordis include:

  • Intracardiac defects
  • Atrial septal defect
  • Ventricular septal defect
  • Tetralogy of Fallot
  • Tricuspid atresia
  • Double outlet right ventricle
  • Non-cardiac malformations
  • Pentalogy of Cantrell
  • Omphalocele
  • Anterior diaphragmatic hernia
  • Cleft palate

Treatment

Due to the rarity and rapid postpartum mortality of ectopia cordis, limited treatment options have been developed. Successful surgeries have been performed, but the mortality rate remains high.

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