Presentation
[…] if the condition was present at the time of inpatient admission. [icd.codes]
We present this case here because our patient had presented with main symptom of cyanosis at 15 years of age which is an unusual presentation. [omicsonline.org]
The severity of disease will determine when the patient presents clinically. Mild pulmonic stenosis will be asymptomatic, and will present at routine physical examination with a systolic ejection murmur. [clinicaladvisor.com]
A heart murmur is the most common sign detected by a physician indicating that a valve problem may be present. [cincinnatichildrens.org]
Stenosis - when the valve doesn't open enough and blocks blood flow Valve problems can be present at birth or caused by infections, heart attacks, or heart disease or damage. [icdlist.com]
Entire Body System
- Atrial Septal Defect
Clinical update: atrial septal defect in adults. Lancet 369, 1244–1246 (2007). 10. Amin, Z. Transcatheter closure of secundum atrial septal defects. Catheter Cardiovasc. Interv. 68, 778–787 (2006). 11. King, T. D., Thompson, S. [nature.com]
NIH: National Heart, Lung, and Blood Institute Atrial septal defect (Medical Encyclopedia) Bicuspid aortic valve (Medical Encyclopedia) Congenital heart defect corrective surgeries (Medical Encyclopedia) Congenital heart disease (Medical Encyclopedia) [icdlist.com]
Septal defects The treatment of ventricular and atrial septal defects depends on the size of the hole. No treatment will be required if your child has a small septal defect that doesn't cause any symptoms or stretch on the heart. [nhs.uk]
Note that the murmur of an atrial septal defect is also midsystolic, however it has a fixed split S2. [healio.com]
Commonly Associated Conditions Tetralogy of Fallot Noonan and LEOPARD syndromes: dysplastic/supravalvular Neurofibromatosis Williams syndrome: supravalvular Alagille syndrome: peripheral Keutel syndrome: peripheral Ventricular septal defect and atrial [unboundmedicine.com]
- Surgical Procedure
Children and adults whose pulmonary valves are still flexible may be treated with a balloon valvuloplasty, a non-surgical procedure in which a catheter — a thin, flexible, plastic tube — is inserted into the heart via blood vessels from the leg. [ucsfhealth.org]
procedures used to correct other heart defects (for example, pulmonary artery banding - a purposeful narrowing of the artery to reduce blood flow to the lungs). [my.clevelandclinic.org]
Open-heart surgical procedures are required for more complex valves, where balloon dilation is not sufficient therapy. [cincinnatichildrens.org]
Examples of these medications include: prostaglandins to improve blood flow blood thinners to reduce clotting water pills to reduce excess fluid in the blood stream pills that prevent irregular heart rhythms A surgical procedure known as a valvuloplasty [healthline.com]
Fontan procedure The Fontan procedure is an open heart surgical procedure used in children with a defect in the structure of the heart and great vessels which are present at birth. [remedyland.com]
- Congestive Heart Failure
Some people with pulmonary valve disorders experience few or no symptoms, but if the condition is not treated it can result in serious complications, including congestive heart failure or a condition called cardiomyopathy, in which the heart muscle becomes [dartmouth-hitchcock.org]
[…] pulmonic valvular stenosis, with pressure gradient greater than 50 mm Hg is more often associated with decreased cardiac output, right ventricular hypertrophy, early congestive heart failure (CHF), and cyanosis. [emedicine.medscape.com]
In one study, 94% patients of mild pulmonary stenosis were asymptomatic, without cyanosis or congestive heart failure [ 12 ]. Valvulotomy has been shown to improve morbidity and mortality and is indicated with these gradients. [omicsonline.org]
[…] disease (CHD) 先天性心疾患 congestive heart failure (CHF)うっ血性心不全 constrictive pericarditis 収縮性心膜炎 continuous murmur連続[性]雑音 contractility収縮性、収縮能 contrast medium造影剤 cor心 cor pulmonale肺性心 coronary angiography (CAG) 冠[状]動脈造影[法] coronary angioplasty冠[状]動脈形成術 coronary [tokyo-med.ac.jp]
Respiratoric
- Exertional Dyspnea
[…] end-systolic volume収縮終(末)期容積、期末容積 end-to-end anastomosis端々吻合 end-to-side anastomosis端側吻合 epicardial心外膜 【形】 epicardium心外膜 ergometer exerciseエルゴメータ運動負荷 escaped beat補充収縮 escaped rhythm補充調律 evoked potential誘発電位 excitation-contraction (E-C) coupling興奮収縮連関 exertional [tokyo-med.ac.jp]
- Tachypnea
[…] sudden death急死〈頓死〉、突然死 superior vena cava (SVC)上大静脈 supraventricular premature contraction(SVPC)上室期外収縮 supraventricular tachycardia (SVT)上室頻拍 symptom 症状 syncope意識消失、失神 systole[心]収縮[期] systolic function収縮機能 systolic murmur (SM)収縮期雑音 t tachycardia 頻拍(脈) tachypnea [tokyo-med.ac.jp]
Cardiovascular
- Heart Failure
Physical Examination Findings of right heart failure can be seen upon physical examination including elevated jugular venous pressure, hepatojugular reflux and lower extremity edema. [healio.com]
Advanced stenosis can lead to heart failure, especially in the twenties and thirties. [remedyland.com]
Key words Neonatal heart failure Intact ventricular septum This is a preview of subscription content, log in to check access. Preview Unable to display preview. Download preview PDF. References 1. [link.springer.com]
The heart can become weakened and stiff, which may lead to heart failure. Symptoms of heart failure include fatigue, shortness of breath, and swelling in the abdomen and legs. [heart-valve-surgery.com]
Presentation See also the separate Heart Auscultation and Heart Murmurs in Children articles. Pulmonary regurgitation is usually asymptomatic unless severe, when it may lead to signs of right heart failure. [patient.info]
- Cyanosis
Case History A 15 year old male presented to us with history of cyanosis for the past 6 years associated with shortness of breath on exertion. [omicsonline.org]
Severe pulmonary valve stenosis is manifested at birth, recalling on clinical pulmonary valve atresia with severe cyanosis and acute heart failure which develops after the closure of the ductus arteriosus. [remedyland.com]
Signs and symptoms of severe defects in newborns include Rapid breathing Cyanosis - a bluish tint to the skin, lips, and fingernails Fatigue Poor blood circulation Many congenital heart defects cause few or no signs and symptoms. [icdlist.com]
- Hypertension
There are also numerous causes of acquired pulmonary artery aneurysms, including infection, vasculitis, pulmonary arterial hypertension, trauma, neoplasm and pulmonary embolism. [pulmonarychronicles.com]
Significant pulmonary regurgitation may also be caused by primary pulmonary hypertension, secondary pulmonary hypertension, infective endocarditis (rare, but may occur in an intravenous drug user or with an atrial septal defect and a large left-to-right [patient.info]
Massive dilatation of the pulmonary artery in association with pulmonic stenosis and pulmonary hypertension. Pulm Circ. 2012 Apr. 2(2):256-7. [Medline]. [Full Text]. Fawzy ME, Hassan W, Fadel BM, et al. [emedicine.medscape.com]
[…] intrinsic (when it is acquired). [1] Pathophysiology [ edit ] The pathophysiology of pulmonary valve stenosis consists of the valve leaflets becoming too thick (therefore not separate one from another), which can cause high pulmonary pressure, and pulmonary hypertension [en.wikipedia.org]
Pulmonary valve prolapse and regurgitation are most often caused by pulmonary hypertension (high blood pressure in the lungs, often as a result of chronic obstructive pulmonary disease). [dartmouth-hitchcock.org]
- Systolic Murmur
Sixteen patients were asymptomatic with pulmonary systolic murmurs, although 6 patients presented with dyspnea. [ncbi.nlm.nih.gov]
Systolic Murmurs - Pulmonary Stenosis You are listening to a typical example of the murmur caused by pulmonary valve stenosis. [med.ucla.edu]
Ejection systolic murmur along the left upper edge of the sternum. Pulmonary ejection click. Delayed second heart sound with severe stenosis. Parasternal thrill and heave. 'A waves' in the JVP. [patient.info]
Physical examination revealed grade 3/6 systolic murmur over the precordium, most prominent in left 2 nd intercostal space. The second heart sound was inaudible. [scirp.org]
In the second and third intercostal space to the left of the sternum there is a prominent thrill and clear harsh systolic murmur. Infundibular stenosis in the presence of murmur is heard in the third and fourth intercostales space. [remedyland.com]
- Tachycardia
(PSVT)発作性上室性頻拍 paroxysmal ventricular tachycardia (PVT)発作性心室頻拍 patent ductus arteriosus (PDA) 動脈管開存[症] percardial friction rub心膜摩擦音 percussion 打診[法]、叩打法〈マッサージの一法〉 percutaneous transluminal coronaryangioplasty (PTCA)経皮的冠[状][動脈]形成[術] percutaneous transluminal [tokyo-med.ac.jp]
Long-term studies have demonstrated that pulmonary regurgitation may lead to progressive right ventricular dilatation, right ventricular dysfunction, exercise intolerance, ventricular tachycardia, and sudden cardiac death. [ 5 ] [patient.info]
Skin
- Pterygium Colli
Noonan’s syndrome ( Figure 11-8C ) 37, 55, 56 is characterized by short stature, webbed neck, pterygium colli, ptosis,hypertelorism, lymphedema, low-set ears, low anterior and posterior hairlines, flat or shield chest, pectus excavatum or carinatum, hyperelastic [clinicalgate.com]
Workup
Axis
- Right Axis Deviation
The electrocardiogram showed a greater degree of right axis deviation than is found in most cases of dome-shaped pulmonary stenosis. [circ.ahajournals.org]
ECG: may show right ventricular hypertrophy, right atrial hypertrophy and right axis deviation. Angiography: may be required in children with multiple cardiac abnormalities. [patient.info]
An ECG can help identify the presence of right ventricular hypertrophy or right axis deviation associated with pulmonic stenosis; however, 40%-50% of patients have a normal ECG, especially if stenosis is mild. [clinicaladvisor.com]
Hypertrophy
- Ventricular Hypertrophy
Right ventricular hypertrophy usually results due to right ventricular pressure overload from the stenotic valve, and this may also include infundibular hypertrophy. [omicsonline.org]
Doppler and ECG analyses showed that delta P did not change significantly similar to immediate gradient after PBPV and right ventricular hypertrophy disappeared progressively 6 months after PBPV. [ncbi.nlm.nih.gov]
Can have a normal heart size or may show evidence of right ventricular hypertrophy. May also show evidence of a dilated pulmonary trunk or a main pulmonary artery. Pulmonary vascularity is often normal in mild cases. [radiopaedia.org]
ECG showing RVH (right ventricular hy- pertrophy) 1 mV = 5 mm standardization. Figure 6. X-ray chest PA view showing main pulmonary artery segment dilatation and RVH (right ventricular hypertrophy). [scirp.org]
Possible outcomes include the development of right ventricular hypertrophy, right ventricular dilation and failure, the development of cyanosis (if an atrial communication is present), exercise intolerance, and reduced cardiac output. [clinicaladvisor.com]
Jugular Venous Pressure
- Prominent A-Wave
ECG of pulmonary valve stenosis Electrical axis deviation to right or right ventricular hypertrophy, prominent P wave. With cardiac catheterization is possible to determine the pressure gradient as the tip of the catheter pass pulmonary valves. [remedyland.com]
Treatment
What is the treatment? Treatment is only needed when the narrowing is of a sufficient severity to be a concern. Some people with only mild or moderate narrowing never need any treatment. [childrensheartcentre.com]
This procedure should be considered as the primary treatment of adult patients with pulmonary valve stenosis. [ncbi.nlm.nih.gov]
After treatment A good result from a balloon valvoplasty or surgery usually means that further treatment is not required. [chd-uk.co.uk]
Many children with congenital heart defects don't need treatment, but others do. Treatment can include medicines, catheter procedures, surgery, and heart transplants. [icdlist.com]
Prognosis
What is the prognosis? The prognosis for this condition is generally good, with survival without complications nearly 100%. In the long term, these children fare well and most have no symptoms and lead normal lives. [childrensheartcentre.com]
Prognosis Pulmonary regurgitation is usually well tolerated in childhood. [patient.info]
Prognosis for pulmonary valve stenosis Patients with moderate stenosis can have a normal life. Bacterial endocarditis can be developed. [remedyland.com]
Mild-pulmonic stenosis has a generally favorable prognosis. Many times, the stenosis will improve or go away completely over the first few years of life. [clinicaladvisor.com]
Etiology
Congenital etiologies cause an abnormal development of distal bulbus cordis secondary to: Rubella embryopathy (most important etiology) Isolated branch pulmonary artery stenosis (stenosis of a sling of the ductus arteriosus) Congenital/genetic syndromes [unboundmedicine.com]
Specific features depend on the etiology, which affects the level at which the obstruction to right ventricular outflow occurs: valvular pulmonic stenosis most common cause level of obstruction thickened and/or structurally abnormal valve leaflets leaflets [radiopaedia.org]
The etiological differential features of valvular pulmonic stenosis are shown in Table 1. 3.2. Clinical Characteristics 3.2.1. [scirp.org]
(Etiology) Pulmonary Valve Stenosis is a narrowing of the pulmonary valve which occurs at birth. The exact cause of the condition is unknown. [dovemed.com]
The most common etiologies are carcinoid syndrome, rheumatic fever, and homograft dysfunction. [4] Years of stenosis can result in subendocardial hypertrophy causing significant outflow obstruction and resulting in right ventricular pressure overload [emedicine.medscape.com]
Epidemiology
Valves made from animal or human tissue (are used for valve replacement), in adults metal valves can be used. [9] [10] Epidemiology [ edit ] The epidemiology of pulmonary valve stenosis can be summed up by the congenital aspect which is the majority of [en.wikipedia.org]
Explore these free sample topics: -- The first section of this topic is shown below -- Basics Description Deformity of pulmonary valve, most commonly congenital, resulting in obstruction of right ventricular (RV) outflow Epidemiology Incidence Predominant [unboundmedicine.com]
Both pulmonary stenosis (PS) and pulmonary regurgitation are often well tolerated for long periods but the overload of the right ventricle has a progressively detrimental effect on right ventricular function. [ 1 ] Epidemiology Pulmonary valve disease [patient.info]
As this process worsens, the asymptomatic adult becomes gradually symptomatic. [11, 12] Epidemiology Occurrence in the United States Approximately 5 out of 1000 infants are born with a congenital cardiac malformation. [5] Cardiac malformation is the most [emedicine.medscape.com]
Epidemiology of ischemic stroke subtypes according to TOAST criteria: incidence, recurrence, and long-term survival in ischemic stroke subtypes: a population-based study. Stroke 32, 2735–2740 (2001). 31. Sacco, R. L. et al. [nature.com]
Pathophysiology
The cause in turn is divided into: valvular, external and intrinsic (when it is acquired). [1] Pathophysiology [ edit ] The pathophysiology of pulmonary valve stenosis consists of the valve leaflets becoming too thick (therefore not separate one from [en.wikipedia.org]
Pathophysiology A systolic pressure gradient between the pumping chamber and its downstream artery is the primary hemodynamic abnormality of stenosis regardless of whether the obstruction is right- or left-sided. [pedsinreview.aappublications.org]
[…] male ( 1 ) African Americans (46.5%), Hispanics (33.1%), and Caucasians (20.4%) ( 1 ) Prevalence 10% of all cases of congenital heart disease In association with other lesions, may be as high as 25–30% of congenital heart disease ( 1 ) Etiology and Pathophysiology [unboundmedicine.com]
[…] analyzed the treatment, quality of life, echocardiography findings, complications, exercise responses, and predisposition to endocarditis with regards to cardiac valvular disease, and pulmonary stenosis was found to be the most benign valvular lesion. [9] Pathophysiology [emedicine.medscape.com]
Prevention
It can help to prevent an infection of the valve. Not everyone with valve stenosis will need this step. [winchesterhospital.org]
Patients with severe or moderate to severe pulmonary valve stenosis require intervention to prevent long term damage to the heart. [pediatricheartspecialists.com]
Endocarditis Prevention If you have PS, you don't need to take antibiotics to prevent endocarditis unless you've had prior endocarditis or you've had valve replacement. See the section on Endocarditis for more information. [heart.org]
The pulmonary valve functions as a one-way valve that allows blood to move from the right ventricle (pumping chamber) into the artery to the lungs and prevents blood from leaking back into the right ventricle. [childrenshospital.org]
Prevention & Risk Assessment Prevention & Risk Assessment What causes pulmonary stenosis? Congenital pulmonary stenosis occurs due to improper development of the pulmonary valve in the first eight weeks of fetal growth. [childrensnational.org]