Cardiac MRI in Pulmonary Hypertension: From Magnet to Bedside



anomalous coronary artery from pulmonary artery :: Article Creator

Anomalous Coronary Origin: The Challenge In Preventing Exercise-related Sudden Cardiac Death

The sudden cardiac death (SCD) of a young athlete is a catastrophic event, particularly in the absence of prodromal warning symptoms. Anomalous coronary origin (ACO) is a well-described cause of cardiac symptoms and SCD, but the diagnosis is usually missed by conventional non-invasive investigations designed to identify myocardial ischaemia. SCD is preventable by correction of the anomaly. A tragic case of a promising young athlete who had underlying ACO and who presented with prodromal symptoms with multiple "negative" investigations is described to highlight the typical clinical features and outline the difficulties encountered in accurate premortem diagnosis.


PLAIN RADIOGRAPHIC DIAGNOSIS OF CONGENITAL HEART DISEASE

PLAIN RADIOGRAPHIC DIAGNOSIS OF CONGENITAL HEART DISEASE

PLAIN RADIOGRAPHIC DIAGNOSIS OF CONGENITAL HEART DISEASE

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2e-1. Total anomalous pulmonary venous connection (Supracardiac). (Legend.)

A. There is cardiomegaly with increased pulmonary arterial markings. There is dilation of both the left and right innominate veins and the right superior vena cava producing the classical "snowman" or "figure of 8" appearance. The superior mediastinum is enlarged secondary to dilation of the right vena cava, innominate vein and ascending vertical vein.

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2e-2. TAPVC (Supracardiac).

A. PA chest radiograph shows mild cardiomegaly, increased pulmonary vascular markings and "snowman" appearance of supracardiac anomalous drainage.

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2e-3. Total anomalous pulmonary venous connection (infradiaphragmatic-obstructed).

A. PA chest radiograph demonstrates increased pulmonary venous pattern with a normal sized heart. There is a right sided pleural effusion. The endotracheal tube is just above the level of the carina.

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2e-4. TAPVC (infradiaphragmatic-obstructed).

A. The heart is normal sized with increased pulmonary venous pattern preferentially in the right upper lobe.

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Total anomalous pulmonary venous connection (TAPVC)

Total anomalous pulmonary venous connection is preferable to anomalous venous return as one may have anomalous drainage in the absence of anomalous connection. Winslow reported the first documented case of PAPVC in 1739 in a patient with a right upper pulmonary vein draining to the superior vena cava.

Anomalous pulmonary venous connection is classified as either partial or total. In TAPVC all the pulmonary veins drain into the right atrium either directly or via a venous channel. In all cases there is an ASD or patent foramen ovale which allows right to left atrial shunting in order to maintain survival. Approximately 1/3 of patients with TAPVC have other associated cardiac lesions including single ventricle, atrioventricular septal defect, transposition of the great arteries, hypoplastic left heart syndrome or patent ductus arteriosus. Many of these patients have heterotaxy syndrome with atrio-visceral situs abnormalities and polysplenia/asplenia (Ivemark syndrome).

Incidence: TAPVC accounts for less than 1% of all cardiac defects. There is a 3:1 male preponderance in infants with infradiaphragmatic TAPVC.

Classification:

  • Type 1: Supracardiac connection (55%); connection to the left innominate vein is the commonest accounting for some 44% of all TAPVC. Typically two anomalous veins from each lung converge directly behind the left atrium and form a common anomalous vertical vein, which passes anterior to the left pulmonary artery and the left main bronchus. Obstruction in this lesion is uncommon. However extrinsic compression may occur in cases where the anomalous vein courses between the left pulmonary artery anteriorly and the left main bronchus posteriorly. Anomalous connection to the right superior vena cava is much less frequent but often associated with heterotaxy syndrome/ multiple complex congenital lesions.
  • Type 2: Cardiac connecion (30%); the pulmonary veins connect at the level of the coronary sinus or in the posterior right atrium near the mid-atrial septum. The anomalous veins may connect via a short channel or multiple openings to the right atrium. The coronary sinus ostium is markedly enlarged although normal in position. One paper reported a 22% incidence of obstruction in this lesion.
  • Type 3: Infracardiac connection (13%); this lesion is virtually always accompanied by some degree of obstructed venous return. The pulmonary veins from both sides converge behind the left atrium and form a common vertical descending vein, which courses anterior to the esophagus and traverses the diaphragm at the esophageal hiatus. This vertical vein may join the portal venous system (80-90% cases) either at the splenic or splenic-superior mesenteric venous confluence. Occasionally the vertical vein may connect directly to the ductus venosus or even the hepatic or inferior vena cava. Obstruction to venous drainage may obviously occur at any point along the abberant path including the esophageal hiatus, the portal venous system or the ductus venosus (discrete). Obstruction may also occur at the level of the hepatic sinusoids (diffuse obstruction). Presentation is generally in the early newborn period.
  • Type 4: Mixed pattern (2%); the commonest pattern of mixed obstruction is drainage of a vertical vein to the left innominate vein and drainage of the right lung either via the right atrium or the coronary sinus. This pattern of anomalous venous connection is generally associated with other major cadiac lesions.

    • What Is Coronary Artery Occlusion?

    Coronary artery occlusion is a partial or total blockage of one of the arteries in your heart. It can cause shortness of breath and chest pain but sometimes doesn't cause symptoms until you experience a complication, like a heart attack.

    Your coronary arteries supply your heart with blood. Coronary artery occlusion is a partial or complete blockage of one of your coronary arteries, which can lead to a heart attack.

    The underlying cause of coronary artery occlusion is usually coronary artery disease (CAD). CAD results from plaque buildup inside your coronary arteries, causing them to narrow. Pieces of this plaque can break off and lead to a blood clot and blockage inside your heart.

    Read on to learn more about coronary artery occlusion, including symptoms, causes, and potential complications.

    Coronary artery occlusion can prevent your heart tissue from receiving enough blood and oxygen. People with a partial blockage may only have symptoms with exercise, whereas people with total occlusion may always have symptoms such as:

    Symptoms of a heart attack

    Coronary artery occlusion can lead to a heart attack. According to the Centers for Disease Control and Prevention (CDC), the major symptoms of a heart attack are:

  • pain in the center or left side of your chest that lasts for more than several minutes or goes away and comes back
  • feeling faint, light-headed, or weak
  • pain in your:
  • pain in one or both of your arms or shoulders
  • shortness of breath

    • Learn more about the early indications of a heart attack.

    Coronary artery occlusion is usually a complication of CAD. Plaque inside your blood vessels, often developing over many years, leads to CAD by narrowing your coronary arteries and reducing blood flow.

    Plaque can also break off your coronary arteries and lead to a blood clot that completely obstructs one of your coronary arteries.

    Another cause of coronary artery occlusion is a coronary artery spasm, which is a temporary constriction (tightening) of the muscles inside your coronary arteries.

    The risk factors of coronary artery occlusion are similar to those of CAD. These risk factors include:

    The leading risk factor for coronary arterial spasms is smoking.

    Doctors can typically make a diagnosis with a test called a coronary angiogram. During this test, your doctor injects a dye into your blood vessels, allowing blood flow to show up on an X-ray.

    Doctors can grade the amount of blood flowing through your coronary artery on the following scale:

    Another diagnostic tool is coronary computed tomography angiography (CCTA). It's a CT scan that looks specifically at your coronary arteries and can estimate the percentage of plaque buildup.

    Other tests you might receive include:

    Treatment for total coronary artery occlusion usually includes one of the following:

  • Percutaneous coronary intervention (PCI): During a PCI, a surgeon inserts a balloon into one of your arteries, moving it through your bloodstream with a long wire until it reaches your heart. The surgeon then expands the balloon in your coronary artery to relieve the blockage before inserting a stent to keep the artery open.
  • Coronary artery bypass graft: A coronary bypass involves taking a blood vessel from another part of your body and using it to create a new pathway for blood to flow around the blockage.

    • Doctors can often treat partial artery occlusion similarly to stable angina. They can prescribe beta-blockers to treat chest pain. Calcium channel blockers and long-acting nitrates are alternative treatment options.

    A doctor will usually recommend lifestyle and dietary changes in combination with these treatments.

    Coronary artery occlusion can lead to a heart attack if your heart doesn't receive an adequate supply of blood. A heart attack can lead to permanent scarring of your heart or death.

    Your outlook with coronary artery occlusion depends on the severity of the blockage. Total occlusion is associated with a worse outlook and higher death rates.

    In a small 2022 study, researchers examined the outcomes of people with chronic total coronary artery occlusion who received medications or PCI at an average follow-up of 56 months. They observed the following:

  • Six of the 48 people who received medications died of cardiovascular disease, and 11 died in total.
  • Three of the 44 people who received PCI died of cardiovascular disease, and 4 died in total.
  • There was no difference in rates of cardiovascular events or quality of life between the groups.

    • Here are some frequently asked questions people have about coronary occlusion.

    What is the survival rate for right coronary artery blockage?

    In a 2021 study, researchers looked at the 10-year mortality rate for people who had received either PCI or a coronary bypass to treat a total occlusion. They found it ranged from 21.4–29.9%, depending on which treatment people received and whether they had a complete occlusion.

    What are the warning signs of clogged arteries?

    Experts often consider CAD to be a silent disease because it may not cause symptoms until a significant blockage causes a heart attack. A type of chest pain called angina is often one of the first symptoms to appear.

    Is a coronary occlusion a heart attack?

    Coronary occlusion is a partial or complete blockage of a coronary artery. This blockage can lead to a heart attack if your heart tissue can't receive enough blood.

    Coronary occlusion is a total or partial blockage of one of the arteries that supply your heart with blood. It can lead to the death of heart tissue and a heart attack.

    Doctors often treat a total coronary occlusion with a coronary bypass or PCI. Partial occlusions may be treatable with medications and lifestyle changes.





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