Pulmonary hypertension (PH) refers to elevated pulmonary artery pressure (PAP) and is commonly defined as mean PAP≥25mmHg. The presence of PH does not indicate any specific pathologic or physiologic cause. This distinction is underappreciated, with consequent confusion in both clinical practice and the medical literature.
Prognosis in PH is predicted by underlying cause, pathophysiology and the interaction between the right heart and pulmonary circulation. The degree of elevation in PAP itself is not the main cause of adverse effects of PH and lowering PAP is not the focus of therapy. This is in contrast to systemic arterial hypertension, where elevated pressure itself causes many subsequent events such as chronic kidney disease and cerebrovascular accident, with therapy being directly aimed at lowering systemic arterial pressure. PH is common among adults with congenital heart disease (CHD). Almost 6% of adults with CHD carry a diagnosis of PH; this subset of patients has a higher risk of hospitalization and death.1 Over 1/3rd of complex ACHD hospitalizations in the United States involve a diagnosis of PH.2 The epidemiology of PH associated with congenital heart disease (PH-CHD), however, is poorly defined, with data based on widely disparate diagnostic techniques and definitions (detailed below). Most importantly, PH is often conflated with pulmonary arterial hypertension (PAH), which refers to PH characterized by elevated PVR but normal pulmonary venous pressure (Table 1). While PH-CHD is stereotypically attributed to high PVR (e.g., Eisenmenger syndrome), elevated pulmonary venous pressure, lung disease, chronic thromboembolism and extrinsic compression of pulmonary vascular structures are all common contributors. Therefore,...
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Additionally, some of the general diagnostic and pulmonary function tests are distinct in emphysema in comparison to chronic bronchitis. In the case of R.S. the arterial blood gas (ABG) values are the following: pH=7.32, PaCO2= 60mm Hg, PaO2= 50 mm Hg, HCO3- = 80mEq/L. R.S.’s laboratory findings are indicative of chronic bronchitis, where the pH and PaO2 are decreased, whereas PaCO2 and HCO3- are increased, when compare to normal indices. Based on the arterial blood gas evaluation, the physician can deduce that the increased carbon dioxide is due to the airway obstruction displayed by the hypoventilation. Furthermore the excessive mucus production in chronic bronchitis hinders proper oxygenation leading to the hypoxia. On the other hand, in emphysema the arterial blood gas values would include a low to normal PaCO2 and only a slight decrease in PaO2 which tend to occur in the later disease stages.
Pulmonary stenosis (PS) - Pulmonary Stenosis causes an obstruction of blood flow from the right ventricle into the pulmonary arteries. This obstruction causes the right ventricle to have more difficulty pumping the oxygen-poor blood received from the vena cava to the lungs in order to pick up the oxygen needed. Therefore causing a decrease in exchange of oxygen in the lungs, as well as a decrease of blood volume to the lungs.
“Hypoplastic left heart syndrome accounts for 9% of all critically ill newborns with congenital cardiac disease, causing the largest number of cardiac deaths in the first year of life.(2) ” HLHS is a severe heart defect that is present at birth. HLHS combines different defects that result in an underdeveloped left side of the heart. This syndrome is one of the most challenging and difficult to manage of all of the congenital heart defects. Multiple portions on the left side of the heart are affected including the left ventricle, the mitral and aortic valve, and the ascending aorta. These structures are greatly reduced in size, or completely nonexistent causing the functionality of the left heart to be reduced, or non-functional all together.
For example, heart and lung transplants. This is a solution if a patient’s hole in the heart cannot be healed or helped with another form treatment. Several different targeted therapies have been done to improve health of ES patients. Disease-targeting therapies have proven to be successful in Idiopathic Pulmonary Arterial Hypertension (IPAH) and have been analyzed to in their effectiveness against ES. Prostanoid therapy in patients with ES has also shown the possibility that therapy may improve oxygen saturation, exercise capacity, and even shows a decrease Pulmonary Vascular Resistance (PVR). This is backed by a study with eight patients that have ES, these results were after 3 months of therapy. Another study is being done in a method called Vasodilator therapy. In these case studies, a drug name prostacyclin improved hemodynamics sufficiently enough to ensure ES patients for surgery to repair the cardiac lesion. Other studies have shown that Phosphodiesterase Type-5 Inhibitors, were used in patients with ES. In these studies, observation over a six-month period allowed the researchers to see improvements in oxygen saturation and cardiopulmonary hemodynamics (Beghetti and Galiè, 2009). Research from these studies is also being combined with different medical treatment to target therapies and is it possible to reverse pulmonary vascular
Many compensatory mechanisms are stimulated in heart failure. These mechanisms involve rising ventricular preload, or the Frank-Starling mechanism, by ventricular dilatation and volume expansion, peripheral vasoconstriction to firstly sustains perfusion to significant organs, myocardial hypertrophy to protect wall strain as the heart expands, kidney sodium and water retention to improve ventricular preload, and start of the adrenergic nervous system, which elevates heart beat and contractile function. The activation of neurohormonal vasoconstrictor systems, which include RAAS, the adrenergic nervous system, and non-osmotic release of vasopressin will control these compensatory mechanisms (Henry & Abraham).
During a physical examination, a specialist may hear a heart murmur which will prompt a referral to a pediatric cardiologist for an analysis. Diagnostic testing will vary by the child’s age, clinical condition, and institutional preferences. Such test may incorporate a chest X-ray, electrocardiogram, echocardiography and cardiac catheterization. A chest X-ray uses unseen X-ray beams to cr...
Tetralogy of Fallot is a congenital heart disease which involves four different heart defects in one. The four different heart defects are a large ventricular septal defect (VSD), Pulmonary Stenosis, Right ventricular hypertrophy, and an overriding aorta. These congenital defects change the normal flow of blood thro...
High blood pressure is called the “silent killer” because it often has no warning signs or symptoms, and many people don’t know they have it. For most patients, high blood pressure is found when they visit their health care provider or have it checked elsewhere. Because there are no symptoms, people can develop heart disease and kidney problems without knowing they have high blood pressure. Some people may experience: bad headache, mild dizziness, and blurry vision. Traditionally, diagnosis of high blood pressure (BP) has relied on consecutive checks of clinic BP over a 2 to 3 month period, with hypertension confirmed if BP remains persistently raised over 140/90 mmHg. This method of diagnosis has significant limitations because the BP measured for an individual patient in a clinic setting may not reflect their BP in day-to-day life. The main concern is that as a result of the “white coat syndrome”, hypertension may be over-diagnosed when checked in the clinic setting; resulting ...
Results: The average age of the initial diagnosis of the disease was at 17 months old and average age of the definite diagnosis was at 44 months old. The initial diagnosis in 85% of the cases was associated with heart murmur. In 27.5% of the cases, VSD had been associated with other cardiac anomalies. Pulmonary artery hypertension was observed in 16.5% of the cases. Fifty nine surgeries were performed on 40 patients.
2017). As the pressure from the volume of blood retaining in the left atrium builds up, it can
My case study encompasses pulmonary embolism and a saddle pulmonary embolism. The patient I chose was a female who had just given birth with no complications during labor, but developed a pulmonary embolism that later on was confirmed to be a saddle pulmonary embolism. Not known to me before this clinical study pulmonary embolism is a leading cause of death among pregnant women in the developed world. A pulmonary embolism is a sudden blockage in a lung artery. The blockage is usually by a blood clot that travels to the lung from the vein in the leg. A clot that forms in one part of the body and travels in the bloodstream to another part of the body is called and embolus (NAT13). Pulmonary embolism is a serious condition that can damage your lung due to lack of blood flow to your lung tissue, which can lead to pulmonary hypertension. Pulmonary hypertension is increased pressures in the pulmonary artery. PE can also cause low oxygen levels in your blood and damage other organs in your body because of a lack of oxygen. If a blood clot is very large it can cause death. A pulmonary embolism can also be called a venous thromboembolism.
Parker, Steve. "Chronic Pulmonary Diseases." The Human Body Book. New ed. New York: DK Pub., 2007.
This condition is close to me because I have the early signs of Primary Pulmonary Arterial Hypertension (PPAH) and I have family members that have died or currently have this condition. Pulmonary arterial hypertension is a progressive disorder characterized by high blood pressure in the pulmonary artery, the blood vessel that carries blood from the heart to the lungs. Pulmonary arterial hypertension occurs when increased pressure cannot fully overcome the elevated resistance and blood flow to the body is insufficient. To overcome the pressure that increases in the pulmonary artery and in the heart chamber that pumps blood into the right ventricle. Treatments for PPH focus on symptoms and include several
As diagnosis may take a greater amount of time to develop or emerge, treatment may not be an option. In some cases the child may require a heart transplant. A young boy by the name of Joseph Gonzalez-Sales, age 10, was a child that required a heart transplant due to dilated cardiomyopathy (Kotojarvi 1). Joseph waited about 2 years to receive his procedure and finally underwent surgery to obtain his new and healthy heart on his 11th birthday (Kotojarvi 1). Other children, in not as consequential cases, can receive some form of treatment that may not require a procedure. Treatment may vary from patient to patient and is directed toward their symptoms and diagnosis (“Pediatric Cardiomyopathy” 7). Unfortunately many drug therapies and surgical
My first patient that was assigned to me was a 50-year-old woman admitted for coughing and nose bleeds. She states that she has been bleeding for the last two to three days. After coughing, she had chest pains that would go away and come back. Her admitting diagnosis was CAD. The patient had past medical history of NSTEMI and chronic diastolic failure, coronary angioplasty, chest pain and hypertension and hyperlipidemia. My assessments went very smoothly. She was able to tell me that she experienced shortness of breath upon exertion. She was not experiencing any pain. Further, I was able to find that she was hypertensive at 148/89. I looked into her issue and discovered that her nose bleeding may be related to HTN because high blood pressure