Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association
MicroRNA In Blood Could Be PAH Marker In Congenital Heart Disease
High levels of a small RNA molecule in the blood may be a marker of pulmonary arterial hypertension (PAH) in people with congenital heart diseases, a new study reveals.
The study, "Expression and Diagnostic Value of miR-3591-5p in Patients with Congenital Heart Disease-Associated Pulmonary Arterial Hypertension," was published in Lung.
PAH is marked by high blood pressure in the vessels that carry blood through the lungs. People with congenital heart disease (CHD), a broad category that encompasses problems with heart structure or function from birth, are at high risk of developing PAH.
MicroRNAs, often abbreviated miRNAs, are small pieces of RNA that regulate gene activity within cells. The impact of these small RNA molecules in human disease is only starting to be understood, but research suggests it could be immense.
"There is evidence that miRNAs are involved in regulating over 30% of the established human genes and nearly all the [disease-driving] processes in humans," wrote researchers at Nantong University in China, who looked for distinct miRNA profiles in blood samples from people with CHD-related PAH. The study included samples from 110 people with CHD. Fifty didn't have PAH, 30 had mild PAH, and 30 had PAH that was moderate to severe. Samples from 40 people who didn't have CHD were included as controls.
PAH marker in people with CHDSignificantly higher levels of a miRNA molecule, dubbed miR-3591-5p, were seen in those with CHD and moderate to severe PAH than in those with only CHD and the controls. It was also higher in those with CHD and mild PAH, and in those with CHD only, than the controls.
Among the PAH patients, data indicated a positive correlation between levels of miR-3591-5p and measures of mean pulmonary artery pressure. In other words, those with higher levels of this miRNA tended to have higher pressure in the blood vessels of their lungs, indicating more severe PAH. Levels of miR-3591-5p also correlated with other disease-relevant measures, such as the amount of resistance in lung blood vessels.
High miR-3591-5p levels, relative to other blood proteins, could accurately identify 41.7% of patients with PAH and 86% of those who didn't have PAH. These findings indicate "miR-3591-5p has predictive and diagnostic value for CHD-PAH," the researchers wrote.
Assessments of biological function indicated miR-3591-5p may play a role in the TGF-beta pathway, a signaling pathway that's been heavily implicated in PAH.
The study "highlights the potential of miR-3591-5p as a biomarker for the diagnosis and assessment of the severity of PAH in patients with CHD," wrote the researchers, who emphasized further research is needed to validate these findings.
While largely focused on miR-3591-5p, the researchers said their analysis identified several other promising miRNA molecules as potential markers of PAH. "It is expected that these differential miRNAs will eventually serve as early diagnostic and prognostic indicators in terms of CHD-PAH," they said.
Understanding Pulmonary Hypertension: Symptoms & Treatments
When most people hear the word hypertension, they think of high blood pressure. While not as common as high blood pressure, pulmonary hypertension, which affects the lungs, can be harder to diagnose, more challenging to treat, and, tragically, often comes with fatal consequences.
Dr. Talal Dahhan, Division Chief for Pulmonary & Critical Care at Baystate Pulmonary explains, "Nearly 3 to 8% of the world's population is affected by pulmonary hypertension (PH). A serious condition brought on by abnormally high pressure in the blood vessels between the lungs and the heart, most cases of PH cannot be cured. But with early detection and proper treatment, it is possible to improve symptoms and slow the disease's progression."
What is Pulmonary Hypertension?While the signs and symptoms of pulmonary hypertension are felt in the lungs, the cause starts in the heart. Dr. Dahhan explains, "In a healthy body, the heart does its job pumping blood around the body and delivering oxygen to organs and tissue. The oxygen gets taken up by the body and the blood cycles back to the heart. When all things are working well, the blood flows back into the heart and travels to the lungs via the pulmonary artery, picks up fresh oxygen, and the whole cycle begins again. However, in cases of PH, the walls of the pulmonary artery become stiff, damaged and narrow and the amount of oxygen going out to the rest of the body drops to dangerously low levels. Sensing the low levels of oxygen, the heart begins to pump harder to push oxygen-low blood to the lungs. Over time, the low level of oxygen in the blood and the added pressure on the heart can lead to serious complications, including heart failure."
What Causes Pulmonary Hypertension?There are a number of conditions and issues that can lead to PH. Once determined, the cause is used to classify a patient's condition by a Group number (Group 1 PH, Group 2 PH, etc.). This grouping information is later used to help identify appropriate treatment options.
Here's a look at the Groups and causes for PH:
Group 1 PH is due to pulmonary arterial hypertension (PAH), a condition in which the tiny arteries in your lung become thickened and narrowed. While PAH can occur due to unknow reasons (what physicians call idiopathic), some common causes include:
Group 2 PH is due to left-sided heart disease, the most common cause of PH in the United States, left-sided heart disease causes blood to back up in the heart, raising the pressure in the pulmonary arteries.
Group 3 PH is due to lung disease, including interstitial lung disease, chronic obstructive pulmonary disease (COPD), certain sleep apneas, pulmonary fibrosis, and emphysema, all of which reduce the flow of blood through the lungs and oxygen uptake.
Group 4 PH is due to blockages in the lungs and arteries, including clots and scars from chronic thromboembolic pulmonary hypertension (CTEPH).
Group 5 PH is due to other diseases and conditions, including blood disorders, kidney disease, inflammatory disorders, and metabolic disorders.
Dr. Dahhan notes that "PH can also be caused by a specific congenital heart called Eisenmenger syndrome. This occurs when there's a hole, or more than one, between the heart chambers, which causes blood to flow incorrectly in the heart, often increasing the blood flow and pressure in the pulmonary arteries."
No matter the cause, Dr. Dahhan notes that PH in all forms is a very serious condition and left untreated can be life threatening.
What Are the Symptoms of Pulmonary Hypertension?According to Dr. Dahhan, "PH symptoms aren't 'all of the sudden' type symptoms. They develop slowly over the years and are often like symptoms of common heart and lung conditions making them easy to miss."
Pulmonary hypertension symptoms include:
Dr. Dahhan adds, "Most people first recognize a problem when they become short of breath when walking. They may be fine on a flat surface but if there's an incline or they're going up stairs, they may find themselves suddenly out of breath. As the disease progresses, they may even feel out of breath when seated. Typically, that's alarming enough to drive someone to seek medical help."
How is Pulmonary Hypertension Diagnosed?As noted, diagnosing PH can be challenging as its symptoms often resemble those of other lung and heart conditions. In addition to performing a physical exam and reviewing your medical history, your doctor may perform the following:
Diagnostic Tests for raising index of suspicion if we have a possibility of Pulmonary HypertensionThe most definite test for diagnosing PH, right heart catheterization measures the blood pressure in the pulmonary arteries. This information identifies where the high pressure exists in your heart and arteries and the specific type (group) of PH the patient has.
What is the Treatment for Pulmonary Hypertension?While there's no cure for most forms of pulmonary hypertension, various treatments are determined by the type of PH. Treatment can help manage symptoms and improve quality of life.
Among the earliest treatments recommended to consider:The next course of treatment options are medications. "There are a number of medication therapies available to help with different types of PH," says Dr. Dahhan, "Depending upon the type of PH a patient has, we may recommend medications to relax or widen blood vessels and arteries, relax muscles in blood vessels, or to increase blood flow. These don't treat PH, but they can treat and help with complications from PH."
A last resort option, surgery may be recommended for patients with CTEPH, however, as Dr. Dahhan notes, not all PH patients are candidates for thromboendarterectomy. But there are procedures to consider in absence of CTEPH. Options include:
Living with pulmonary hypertension can be challenging, but with proper management and care, it is possible to maintain a good quality of life. "Early diagnosis and treatment are crucial," says Dr. Dahhan. "If you are experiencing persistent shortness of breath or other symptoms, reach out to your doctor to schedule an appointment as soon as possible. The more information you can bring to the appointment—how long have you had symptoms, what makes them worse or better, etc.—the easier it will be to identify if PH is behind your issue and the faster you can begin treatment."
641 Morbidity Boss Associated With Nutritional State In Congenital Cardiac Surgery
Background and aim: Nutritional condition is a very important factor in cardiac surgery morbidity outcome. Study focused in relation among nutritional state and morbidity boss in a group of patients led to surgical correction for cardiac malformation.
Methods: Under ambispective serial cases design, we realized a study with a group of patients go cardiac surgery by congenital cardiopathy in four years. By check patients files obtained antrophometric measures and calculated mass corporal index and Z value to establish nutritional condition, as well as, the boss or morbidity decided for alterations in clinical and laboratory test associated with; hypertension and renal, pulmonary, cardiac, hepatic and acid-base dysfunction.
Results. Were included 229 patients' files with an average age of 7.35 years. Predominated not complex cardiopathies (83%) and cyanogen type (85.20%). Nutritional state in 193 (84.30%) patients' was normal, with morbidity incidence of 58.10%. Existed difference in morbidity by hypertension on having compared nutrition state (p=0.040), weight (p=0.010), Z value (p=0.001) and IMC (P=0.020); by renal dysfunction in height (p=0.001) and IMC 8P=0.000); hepatic dysfunction in nutritional state (p=0.000) and IMC (P=0.002). Also existed difference between type of cardiophaty and complexity for; hypertension (p=0.000) and pulmonary (p=0.000), cardiac (p=0.000), hepatic (p=0.002) and acid-base (p=0.000) dysfunction.
Conclusions: Nutritional state of patients with congenital cardiophaty have an important role for after surgery morbidity. A better nutritional state control must be tried before surgery for adaptation to trauma metabolic response in this type of patients.
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