What is Heart Disease?
Heart disease or cardiovascular disease (CVD) is a group of disorders involving the heart and blood vessels (1). These ailments are related to atherosclerosis, the buildup of plaques in the arteries’ walls. (2)
The buildup of plaque narrows the arteries, hence making it difficult for the blood to flow. When a blood clot blocks the blood flow, it causes a heart attack or stroke (3).
Strokes and heart attacks are caused by the use of tobacco, an unhealthy diet, obesity, lack of physical activity, harmful alcohol use, hypertension, diabetes, and hyperlipidemia, or high levels of cholesterol in the blood (4).
What are the Different Heart Diseases?
According to the World Health Organization, CVDs include (5):
- Coronary heart disease – involves the blood vessels that supply the heart
- Cerebrovascular disease – involves the blood vessels that supply the brain
- Peripheral arterial disease – involves blood vessels that supply the arms and legs
- Rheumatic heart disease – damage to the heart and its valves due to rheumatic fever
- Congenital heart disease – malformations in the structure of the heart upon birth
- Deep vein thrombosis and pulmonary embolism – blood clots in the leg veins that can move to the heart and lungs
Symptoms of Heart Diseases
Often, diseases that involve the blood vessels have no symptoms, but a stroke or heart attack could signal that a patient has underlying conditions of the blood vessels.
Meanwhile, patients should be on the lookout for symptoms of rheumatic disease, which include shortness of breath, fatigue, irregular heartbeats, chest pain, and fainting. This disease is caused by rheumatic fever (6).
A person who has fever, pain and swelling of the joints, nausea, stomach cramps, and has been vomiting may have a rheumatic fever (7).
Treatment of Heart Diseases
Some treatments include (8):
- Coronary angioplasty – inserting a balloon to stretch a narrow or blocked artery
- Coronary artery bypass graft surgery
- Heart valve surgery
- Carotid endarterectomy – surgery in the internal carotid artery
- Medications – clot busters that break up the clots blocking blood flow
Can CBD Oil Help Treat Heart Disease?
Studies have found that cannabidiol (CBD), the non-psychoactive cannabinoid component of the cannabis plant, can help treat some heart diseases.
According to a study in 2010 (9), CBD has antioxidant, anti-inflammatory, and anti-apoptotic (prevents cell death) properties that contribute to heart health. The study also concludes that CBD helps suppress arrhythmia, an abnormality where the heart’s rhythm is either too slow, too fast, or irregular.
Another study conducted in 2020 (10) has found that CBD has cardioprotective properties that can help in treating chronic heart failure.
A 2012 study (11) also reports that the administration of CBD oil helps protect patients against ischemia-reperfusion injury, which happens when blood supply returns to tissues after a period of lack of oxygen (ischemia). It also reduces cardiac dysfunction associated with diabetes.
CBD also helps alleviate symptoms of hypertension, as well as a hemorrhagic and cardiogenic shock (12).
CBD may be administered to animals like cats and dogs to help treat their heart conditions, too. All humans and animals (except insects) (13) have an endocannabinoid system (ECS) that works the same way; hence, if CBD is potent on one species, it is likely to have positive effects on other animal species, too.
The ECS is essential in maintaining the body’s homeostasis, including functions of the brain, endocrine, and immune systems, among others. (14)
The U.S. Food and Drug Administration has not approved the use of CBD, so there is no standard dosage for it. When administering whether to pets or humans, it is recommended to begin with a small dose of CBD. If no adverse effects are monitored, the dosage can be gradually increased.
Full-spectrum CBD products are more recommended over the other CBD types, namely broad-spectrum CBD and CBD isolate.
CBD isolate contains pure CBD only while broad-spectrum contains CBD and terpenes (the other parts of a cannabis plant), but without tetrahydrocannabinol (THC, the psychoactive component of the cannabis plant).
Full-spectrum CBD products meanwhile make use of all the cannabinoids that can be found in the cannabis plant, including THC. These are used to achieve the entourage effect, in which all the active ingredients work in synergy to give maximum therapeutic benefits to people who take these.
It is best to consult with a doctor or veterinarian before using CBD, especially since full-spectrum CBD products contain doses of THC.
Various heart diseases can lead to a stroke or heart attack. Aside from medications, most of the treatments for these cardiovascular problems are non-preventive.
CBD oil has cardioprotective, antioxidant, anti-inflammatory, and anti-apoptotic properties that have excellent effects on the heart.
Because humans and animals have ECS, CBD can be useful on pets like cats and dogs, too.
It is recommended that full-spectrum CBD products be used for maximum therapeutic effect.
Before adding CBD to a patient or a pet’s medication regimen, it is best to consult with a doctor or veterinarian first.
- “Cardiovascular Diseases (CVDs).” World Health Organization, World Health Organization, 17 May 2017, www.who.int/news-room/fact-sheets/detail/cardiovascular-diseases-(cvds).
- “What Is Cardiovascular Disease?” Www.heart.org, www.heart.org/en/health-topics/consumer-healthcare/what-is-cardiovascular-disease.
- World Health Organization. (2017 May 17). op. cit.
- Heart.org. op. cit.
- Walsh, Sarah K, et al. “Acute Administration of Cannabidiol in Vivo Suppresses Ischaemia-Induced Cardiac Arrhythmias and Reduces Infarct Size When given at Reperfusion.” British Journal of Pharmacology, Blackwell Publishing Ltd, July 2010, www.ncbi.nlm.nih.gov/pubmed/20590615.
- Lozano, Omar, et al. “CARDIOPROTECTIVE EFFECT OF CANNABIDIOL IN A NON ISCHEMIC MODEL OF HEART FAILURE.” JACC, Journal of the American College of Cardiology, 26 Mar. 2020, www.onlinejacc.org/content/75/11_Supplement_1/705.
- Stanley, Christopher P et al. “Is the cardiovascular system a therapeutic target for cannabidiol?.” British journal of clinical pharmacology vol. 75,2 (2013): 313-22. doi:10.1111/j.1365-2125.2012.04351.x
- Pacher, P et al. “Cardiovascular pharmacology of cannabinoids.” Handbook of experimental pharmacology ,168 (2005): 599-625. doi:10.1007/3-540-26573-2_20
- Silver, Robert J. “The Endocannabinoid System of Animals.” Animals : an open access journal from MDPI vol. 9,9 686. 16 Sep. 2019, doi:10.3390/ani9090686
- Letscher, Emily. “The Endocannabinoid System, Our Universal Regulator.” Journal of Young Investigators, Journal of Young Investigators, 1 June 2018, www.jyi.org/2018-june/2018/6/1/the-endocannabinoid-system-our-universal-regulator.
7 May 2020
CLINICAL AND RADIOLOGICAL EVALUATION OF TER HAAR SYNDROME: CASE REPORT OF A PATIENT WITH EXTREME LONGEVITY
Eda Haznedaroglu, MD1, Ilknur Tanboga, MD1, Sarkis Sozkes, MD2
1Department of Pediatric Dentistry, Dentistry Faculty, Marmara University, Istanbul, Turkey
2 Department of Biomaterials, Biomedical Engineering, Namik Kemal University, Turkey
Ter Haar syndrome (THS) is one of the most deleterious disorders known in medicine today. Cases of THS are rare in the literature, and dental treatment for these children has never before been reported. Here we discuss the preventive and conservative treatment we provided to a patient with THS, and review the literature on the subject.
Ter Haar syndrome (THS) was first described in 19821 and is characterized by congenital glaucoma, craniofacial abnormalities, hypertelorism, kyphoscoliosis, skeletal dysplasia, congenital heart defects, and developmental delay. THS has an autosomal recessive inheritance pattern. The cardiac components of the disease are the most significant, as they are the primary causes of mortality.1–4 Typical radiologic findings with this syndrome are bony sclerosis of base structures, abnormal dental alignment, sclerosis and irregularity of clavicles and ribs, described as ‟band‐like” deformity; and cortical irregularity of tubular bones, with metaphyseal flaring and diaphyseal bowing. The flat bones are broadened, particularly the iliac crest and vertebral bones.5 Cases of THS are rare in the literature, and dental treatment for these children has never been reported. Disabled individuals’ physical, cognitive, and behavioral limitations require modification of standard preventive and conservative dental practices, including the choice of materials and techniques used. On World Health Day at the World Health Organization in 1994, a new method for treating dental caries was presented for developing countries. This approach, called atraumatic restorative treatment (ART), was introduced as a potentially viable means of providing restorative and preventive care. The ART approach uses glass ionomer cement (GIC) as a restorative material and fissure sealant in permanent and primary teeth, and combines both preventive and restorative procedures.6 GICs represent an alternative to resin‐based materials, especially when resins are contraindicated, as in the clinical treatment of children with deeply pitted or fissured primary molars, or permanent first or second molars that have not fully emerged and whose isolation can be diﬃcult, such as in young children and the disabled.7 We have provided preventive and conservative treatment to the longest‐lived patient with THS. In this report, we present his case and review the associated literature.
In July 2003, a 6‐year‐old boy was referred to the Department of Pediatric Dentistry of Marmara University in Istanbul, Turkey. The patient presented with discomfort on eating. He was genetically diagnosed with THS immediately aGer his birth. Clinical history revealed that he was born full term as the fiGh child, with a birth weight of 2000 g. Although the boy had 2 living healthy sisters, the family experienced the loss of 3 children, possibly from heart failure at the ages of 4, 3, and 1. All 3 children had similar syndromic face overview, but none of them had genetic testing to define any possible syndromes. The patient’s parents are relatives―his mother and father are cousins. The mother used acetylsalicylic acid (aspirin) during pregnancy for hypertension.
We report on this patient with congenital glaucoma, congenital heart defects, brachycephaly with flat occiput, large anterior fontanel, hypertelorism, inguinal hernia, kyphoscoliosis, megalocornea, exophthalmos, large ears, short hands and feet, flexion deformity of the fingers, and clubfeet (Figure 1). He had a congenital mitral prolapse, severe mitral deficiency, pulmonary deficiency, severe pulmonary hypertension, and pericardial eﬀusion. There was no mental retardation. In his history, he had trabeculectomies to decrease his intraocular pressure caused by glaucoma when he was 2 days old (however,he had right‐eye blindness); an orchiectomy for cryptorchidism when he was 4 years old, and frequent respiratory tract infections. He received a mitral valve repair at 6 years of age.
Intraoral finding was hyperplastic gingiva (Figure 2). Radiographs showed carious involvement of the pulp of tooth 54. Radix was present in 52, 62, 64, 73, 74, 83, and 84. Dentin caries were found in 55, 65, 75, and 85. Lack of congenital germs was also noted in 35 and 45. We
observed rapid bone resorption at his six months checkup (Figure 2). The surface protection material (Fuji Triage, GC Europe NV) was placed on caries‐free first permanent premolars, and restorative GIC material (Fuji IX, GC Europe NV) was used for teeth with caries (Figure 3).
Glass ionomer capsules in the Fuji Triage and IX kits were mixed according to the manufacturer’s instruction and a ‟pressed finger” (according to ART)6 was used to spread GIC to seal the pits, fissures, and cavities. The pits and fissures were slightly overfilled. To enhance adherence and penetration of the material, a gloved finger with petroleum jelly was used to push the material into pits and fissures. The excess was removed by moving the finger sideways aGer a few seconds. Topical fluoride varnish was applied in 6‐month intervals for prevention. Treatment was preventive and conservative only, and the patient lived free of acute symptoms for 6 years. Unfortunately, he died of cardiac pathology at age 13 years.
Originally considered a form of Melnick‐Needles syndrome (MNS), THS is now recognized as a separate entity that, in addition to the Melnick‐Needles phenotype, includes congenital heart defect and glaucoma.1,2 In 1982, ter Haar et al reported on 3 patients in a family with an autosomal recessive form of MNS. This diagnosis was based on the striking resemblance of the craniofacial and skeletal characteristics seen in these 3 patients to those seen in MNS. A remarkable diﬀerence is the mode of inheritance: classical MNS has X‐linked dominant inheritance, with mortality in aﬀected males.1,8 THS occurs more frequently in males, whereas the male‐to‐ female ratio in MNS is 1:7. Another noteworthy diﬀerence is prognosis: MNS is usually a benign condition, whereas all patients in the family described died of cardiovascular anomalies at an early age.2
Both syndromes are extremely rare―only 11 patients with THS and fewer than 60 patients with MNS have been reported. The largest series of THS includes the 3 patients defined by ter Haar et al.1‐3 The family history of our patient revealed the deaths of 3 siblings with similar facial appearance and symptoms, suggesting that their heart failure was most likely due to THS, but we were not able to consider genetic testing.
When caregivers and parents were questioned, it was clear that they had paid no attention to the oral hygiene of these children. Grannath and Widenheim9 and Nicolai and Tesini10 also have reported that caregivers are not familiar with how to maintain oral hygiene in children with special needs. Therefore, the main focus of dental care for these patients should be promotion of oral health, early diagnosis, and intervention when problems occur. To achieve this, the dental staﬀ must have good communication skills and be able to support the families and encourage them to maintain daily oral health care. However, there is very limited, even insuﬃcient, research on eﬀective ways to prevent dental caries and periodontal disease in patients with disabilities.
This is the first case in the literature of a patient with THS undergoing dental treatment, the 11th case whose cardiac pathology was corrected by Barlas and colleagues,3 and the first male with THS to survive 13 years.
- ter Haar B, Hamel B, Hendriks J, de Jager J. Melnick‐ Needles syndrome: indication for an autosomal recessive form. Am J Med Genet. 1982;13:469‐477.
- Hamel BCJ, Draaisma JMT, Pinckers AJLG, de Jager J. Autosomal recessive Melnick‐Needles syndrome or ter Haar syndrome? Report of a patient and reappraisal of an earlier report. Am J Med Genet. 1995;56:312‐316.
- Barlas S, Ugurlucan M, Ayhan Y, et al. Mitral valve repair in a patient with ter Haar syndrome. J Card Surg. 2006;21:488‐491.
- Dundar M, Saatci C, Tasdemir S, et al. Frank‐ter Haar syndrome with unusual clinical features. Eur J Med Genet. 2009;52:247‐249.
- Krajewska‐Walasek M, Winkielman J, Gorlin RJ. Melnick‐Needles syndrome in males. Am J Med Genet. 1987;27:153‐158.
- Frencken JE, Pilot T, Songpaisan Y, Phantumvanit P. Atraumatic restorative treatment (ART): rationale, technique, and development. J Public Health Dent. 1996; 56:135‐140.
- Ashwin R, Arathi R. Comparative evaluation for microleakage between Fuji‐VII glass ionomer cement and light‐cured unfilled resin: a combined in vivo in vitro study. J Indian Soc Pedod Prev Dent. 2007;25:86‐87.
- Neou P, Kyrkanides S, Gioureli E, Bartsocas CS. Melnick‐Needles syndrome in a mother and her son. Genet Couns. 1996;7:123‐129.
- Grannath KC, Widenheim J. Evaluation of information on dental health care at child health centers. Factors in caries prevention―opinions of dental personnel and their relation to parental attitudes. Acta Odontol Scand. 1994;52:266‐270.
- Nicolai AB, Tesini DA. Improvement in the oral hygiene of institutionalized mentally retarded individuals through training of direct care staﬀ: a longitudinal study. Spec Care Dentist.1982;2:217‐221.
EISENMENGER SYNDROME: PULMONARY HYPERTENSION RESULTING IN A RIGHT‐TO‐LEFT CARDIAC SHUNT
Oliver De Neini, BSc
Eisenmenger syndrome is a complication of uncorrected congenital heart defects that cause leG‐to‐right shunting. Increased pulmonary vascular resistance develops over time, leading to pulmonary vascular injury and pulmonary hypertension, and reversing the shunting to right‐to‐leG. This cardiac shunt reversal allows deoxygenated blood into the systemic circulation, resulting in symptoms of chronic hypoxemia. Signs of chronic hypoxemia, such as cyanosis and digital clubbing, are present, and heart sounds and electrocardiogram findings are variable, depending on the
underlying structural defect. Diagnosis of Eisenmenger syndrome is suggested by chest radiography and electrocardiography, and confirmed by echocardiography and cardiac catheterization. Once pulmonary hypertension has developed, surgical repair of the underlying defect is no longer possible. Treatment is limited to supportive measures, with transplant the only curative option.
Congenital heart defects are among the most common birth defects, aﬀecting approximately 1% of live births.1 A congenital heart defect that causes leG‐to‐right shunting of blood, resulting in changes to the endothelial lining and muscle tissue, hence increases the pressure in the pulmonary circulation. If leG untreated, the defect may result in severe pulmonary vascular obstruction, eventually leading to a bidirectional cardiac shunt, and even complete reversal of leG‐to‐right shunting to right‐to‐leG. This cardiac shunt reversal is known as Eisenmenger syndrome (ES), and an estimated 8% of patients with congenital heart defects are at risk of developing the condition.1 The structural anomalies that, if untreated, lead to ES include ventricular septal defect (VSD), persistent truncus arteriosus, transposition of the great arteries, and, to a lesser extent, atrial septal defect
and patent ductus arteriosus. ES represents the most severe form of pulmonary hypertension, and development of the condition is dependent on several factors, including the site and size of the defect, and the presence of any coexisting cardiac anomalies. Development of ES can be prevented by early
closure of the underlying structural anomaly, but once the condition develops, surgical repair is contraindicated.2 The incidence of ES has markedly decreased in developed countries, due to the early diagnosis and repair of causative anomalies. This review outlines the underlying pathophysiology of ES, and describes the clinical presentation, diagnosis, and management of the condition.
In 1897, the German physician Victor Eisenmenger first described the condition aGer the autopsy of a 32‐year‐ old man who had dyspnea and progressive cyanosis, and who subsequently died of massive hemoptysis.3 The autopsy revealed a large VSD and severe pulmonary vascular disease. In 1958, Paul Wood determined that ES resulted from systemic pulmonary hypertension as a consequence of elevated pulmonary vascular resistance (PVR), leading to reversed cardiac shunting through a VSD.4 Since then, the definition of ES has been expanded to include reversed cardiac shunting through any communication between the pulmonary and systemic circulations.
In the normal heart, the systemic circulation is the higher pressure circuit, with systemic vascular resistance (SVR) at 700 to 1400 dyn•sec•cm‐5, and the pulmonary circulation is the lower pressure circuit, with PVR at 20 to 130 dyn•sec•cm‐5.5 Therefore, in the instance of one or more structural defects resulting in communication between the pulmonary and systemic circulations, a volume of blood flows down the pressure gradient from leG‐to‐right (ie, from the high‐pressure systemic circulation to the low‐pressure pulmonary circulation). As a result of the volume of this shunt entering the right side of the heart, the stroke volume of the right ventricle is increased. A combination of the chronic increased right ‐sided blood pressure and increased blood volume results in mechanical injury of the pulmonary capillaries.5
According to current thinking, endothelial cell dysfunction results from the shear and mechanical injury caused by the increased right‐sided blood pressure.5 Endothelial cell dysfunction leads to increased production of vasoconstrictive agents, such as endothelin, as well as decreased production of vasodilatory agents, such as prostacyclin. In addition to the endothelial cell dysfunction, there is an increased synthesis of growth factors and cytokines that result in the migration and replication of vascular smooth muscle. These vascular alterations involve the entire arterial tree, where proliferation of myointimal cells and smooth muscle cells promotes thickening of the vascular intima and media, and a subsequent reduction in luminal diameter, as well as a thickening of the internal and external elastic membranes. A combination of the narrowed pulmonary vascular lumen and the decreased compliance of the vessels due to medial hypertrophy increases the PVR, and results in compensatory right ventricular hypertrophy (RVH). Eventually, this combination of elevated PVR and RVH leads to an increase in right heart pressure that is suﬃcient to reverse the leG‐to‐right cardiac shunt. As such, blood now flows through the structural defect from right‐to‐leG, causing deoxygenated blood to enter the leG side of the heart and flow out through the aorta, leading to hypoxemia and cyanosis (Figure). The resulting decrease in oxygen saturation culminates in the secondary polycythemia commonly seen in ES.
SIGNS AND SYMPTOMS
The symptoms associated with ES do not usually present until age 20 to 40 years,7 except in the case of pregnancy. The hemodynamic changes corresponding to pregnancy serve to further the deterioration of the intracardiac shunt, increasing the risk to both mother and child.8
Many of the symptoms of ES are specifically related to either the pulmonary hypertension or the resulting chronic hypoxemia that are typical of the disease. Thus, patients with ES frequently present with dyspnea on exertion, cyanosis, fatigue, syncope, atrial and ventricular arrhythmias, and digital clubbing. Angina is also oGen present, due to decreased myocardial oxygenation and
increased right ventricular strain. Hemoptysis is a late symptom, and may be caused by either hypoxemia or pulmonary vascular injury (PVI). Rarely, symptoms of right heart failure may be present, such as peripheral edema, hepatomegaly, and jugular venous distention.
On examination, central cyanosis is evident, and a holosystolic murmur of tricuspid regurgitation may be heard at the lower leG sternal border. A loud, single, second heart sound is a frequent finding.
Laboratory findings of ES are consistent with chronic hypoxemia. As such, elevated hematocrit (>55 %), increased hemoglobin levels (commonly in excess of 20 g•dL‐1), prolonged bleeding times, and decreased oxygen saturation are almost always seen.9 Increased turnover of erythrocytes may also be seen as microcythemia, hyperuricemia, and hyperbilirubinemia.
A diagnosis of ES may be suggested by chest radiography. In the early stages of the disease, chest radiography resembles the typical appearance of increased pulmonary flow, namely right ventricular or biventricular enlargement, right atrial or biatrial enlargement, and a dilated pulmonary trunk. With more advanced PVI, a normal cardiac shadow is seen, with a dilated pulmonary trunk and pulmonary arteries. Diminished peripheral pulmonary vasculature may also be seen in severe disease.
Electrocardiography will resemble that found in RVH, such as frontal plane QRS right axis deviation, tall monophasic R‐waves in RV leads (V3R and V4R), deep S‐ waves in LV leads (V3‐V9), and ST‐segment and T‐wave changes directed opposite to QRS direction.10 There also will be specific electrocardiograph changes associated with the causative structural heart defect. Confirmation of the diagnosis is made by either echocardiography or cardiac catheterization. Echocardiography with Doppler studies is useful for revealing the underlying structural defect, as well as any coexisting abnormalities, and for determining the direction of intracardiac blood flow. Cardiac catheterization allows for examination of the internal cardiac structure, measurement of pulmonary arterial pressures, and calculation of PVR. Cardiac catheterization also permits the exclusion of other causes of pulmonary hypertension.2
As well as being one of the presenting symptoms, one of the most frequently seen complications associated with ES is hemoptysis, arising from either hypoxemia or PVI. Although the hemoptysis is usually mild and self‐limiting, on rare occasions it can be massive and the cause of sudden death among this patient population, as observed in the case with which Eisenmenger first described the condition.3 As well as chronic hypoxemia resulting from pulmonary hypertension, patients also have an underlying congenital structural abnormality, and thus are vulnerable to several bleeding disorders commonly associated with congenital heart defects, such as thrombocytopenia, abnormal fibrinolysis, and acquired type II von Willebrand factor abnormalities.12 As a result, abnormal or prolonged bleeding is of great concern, especially as any loss in blood volume will lower the SVR, and further the severity of the right‐to‐leG shunt. Patients are also at risk of developing brain abscess or endocarditis. Another frequently seen complication of ES is renal dysfunction as a direct result of cyanosis, hypoxemia, and polycythemia. Hence, the extent of renal dysfunction is directly related to the severity of these factors, and ranges from decreased glomerular filtration rate (from reduced blood flow) to nephrotic syndrome. The secondary polycythemia commonly seen in this condition oGen results in hyperviscosity, and thus there is a serious danger of thrombotic events, such as pulmonary embolism or cerebrovascular accident, from
the altered hemodynamic state. Polycythemia may also result in hyperuricemia, gout, cholelithiasis, or hypertrophic osteoarthropathy, and the severity of these complications is correlated with the extent of erythrocytosis.13
Ideally, corrective surgery to repair the underlying defect should have been performed earlier to prevent ES. Surgical repair of the underlying structural defect is not possible once pulmonary hypertension has developed.2 Closure of the defect, and hence fixation of the shunt, causes increased right ventricular pressure, and thus furthers right heart failure. Surgical repair can only be considered if significant leG‐to‐right shunt flow remains, and if the pulmonary circulation is shown to be responsive to vasodilator therapy. Drugs that may lower pulmonary arterial pressure, such as prostacyclin antagonists,14 endothelin anatagonists,6 and nitric oxide enhancers,15 are being studied for use in patients with pulmonary hypertension. Other treatment may also include aspirin to reduce the risk of thrombosis, allopurinol to treat associated gout, and digitalis to reduce the symptoms of right heart failure. Current National Institute for Health and Care Excellence guidelines state that all patients be given endocarditis prophylaxis prior to any surgical procedure likely to cause bacteremia.16 In addition to medical therapy, supportive treatment including the avoidance of factors that may worsen the condition (such as volume depletion, high altitudes,17 and pregnancy7) and the use of supplemental oxygen may be beneficial.18 Phlebotomy may also be necessary to maintain the hemoglobin level at <20 g•dL‐1, to minimize the complications associated with hyperviscosity.2
Transplant is the only specific treatment option for patients with ES, but due to the associated risks, it is reserved for those with severe symptoms and an unacceptable quality of life. There are 2 options for transplant; either a bilateral lung transplant or a heart‐ lung transplant. If the cardiac defect is simple and fixable, and the PVI has been shown to be irreversible, then a bilateral lung transplant with surgical repair of the cardiac defect is possible. However, if the cardiac defect is
complex, then a heart‐lung transplant is necessary. A good prognosis can be obtained with a transplant, with 5‐ year and 10‐year survival rates for those receiving both heart and lungs estimated to range from 30% and 50%, to 50% and 70%, respectively.19,20 As with any transplant procedure, the main complications are infection and rejection, and there is the possibility of obliterative bronchiolitis.20
Eisenmenger syndrome, as the end result of numerous untreated congenital heart defects, represents a unique and challenging pathophysiology. Patients do not usually present until the third decade of life, and the main symptoms are those of chronic hypoxemia; thus, ES presents as a multisystem disease. Although advances in medical understanding, and early diagnosis and repair of congenital heart defects, have decreased the incidence of the condition, treatment options for patients with ES remain severely limited. Further research into the treatment of pulmonary hypertension is necessary, to enable safe repair and an eﬀective cure of the cardiac anomaly present in patients with ES.
- Vongpatanasin W, Brickner ME, Hillis LD, Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med. 1998;128(9):745‐755.
- Oechslin E, Mebus S, Schulze‐Neick I, et al. The adult patient with Eisenmenger syndrome: a medical update aGer Dana Point Part III: specific management and surgical aspects. Curr Cardiol Rev. 2010;6(4):363‐372.
- Eisenmenger V. Die angeborenen defecte der kammerscheidewand des herzens. Z Klin Med. 1897;32:1‐ 28.
- Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J. 1958; 2(5099):755‐762.
- Wright JL, Levy RD, Churg A. Pulmonary hypertension in chronic obstructive pulmonary disease: current theories of pathogenesis and their implications for treatment. Thorax. 2005;60(7):605‐609.
- Cacoub P, Dorent R, Maistre G, et al. Endothelin‐1 in primary pulmonary hypertension and the Eisenmenger syndrome. Am J Cardiol. 1993;71(5):448‐450.
- Onat T, Ahunbay G, Batmaz G, Celebi A. The natural course of isolated ventricular septal defect during adolescence. Pediatr Cardiol. 1998;19(3):230‐234.
- Weiss BM, Hess OM. Analysis of pulmonary vascular disease in pregnant women. J Am Coll Cardiol. 1999;4 (5):1658.
- Donohue CM. Eisenmenger syndrome: a case study. Am J Crit Care. 2001;10(2):117‐120.
- Nauster TD, Stites SW. Diagnosis and treatment of pulmonary hypertension. Am Fam Physician. 2001;63 (9):1789‐1799.
- Moceri P, Dimopoulos K, Liodakis E, et al. Echocardiographic predictors of outcome in Eisenmenger syndrome. Circulation. 2012;126(12):1461‐1468.
- Hopkins WE, Ochoa LL, Richardson GW, Trulock EP. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant. 1996;15 (1 pt 1):100‐105.
- Henriksson P, Varendh G, Lundstrom NR. Haemostatic defects in cyanotic congenital heart disease. Br Heart J. 1979;41(1):23‐27.
- Rosenzweig EB, Kerstein D, Barst RJ. Long‐term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation. 1999; 99(14):1858‐ 1865.
- Michelakis E, Tymchak W, Lien D, et al. Oral sildenafil is an eﬀective and specific pulmonary vasodilator in patients with pulmonary arterial hypertension: comparison with inhaled nitric oxide. Circulation. 2002;105(2):2398‐2403.
- National Institute for Health and Care Excellence website. Prophylaxis against infective endocarditis: antimicrobial prophylaxis against infective endocarditis in adults and children undergoing interventional procedures. March 2008. https://www.nice.org.uk/guidance/cg64. Accessed February 15, 2015.
- Das BB, Wolfe RR, Chan KC, et al. High‐altitude pulmonary edema in children with underlying cardiopulmonary disorders and pulmonary hypertensionliving at altitude. Arch Pediatr Adolesc Med. 2004; 58(12):1170‐1176.
- Sandoval J, Aguirre JS, Pulido T, et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med. 2001;164(9):1682‐1687.Stoica SC, McNeil KD, Perreas K, et al. Heart‐lung transplantation for Eisenmenger syndrome: early and long‐term results. J Ann Thorac Surg. 2001;72(6):1887‐1891.
- Waddell TK, Bennett L, Kennedy R, et al. Heart‐lung or lung transplantation for Eisenmenger syndrome. J Heart Lung Transplant. 2002;21(7):731‐737.