• Users Online: 513
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
CURRICULUM IN CARDIOLOGY - CASE DISCUSSION
Year : 2018  |  Volume : 4  |  Issue : 3  |  Page : 218-223

Bedside case discussion


1 Department of Cardiology, AIIMS, New Delhi, India
2 Department of Medicine, AIIMS, New Delhi, India

Date of Web Publication11-Jan-2019

Correspondence Address:
Dr. Javaid Ahmed
Department of Cardiology, AIIMS, New Delhi
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpcs.jpcs_41_18

Rights and Permissions
  Abstract 

A young boy presented with cyanosis and shortness of breath since childhood. Clinically, he has a single second sound, a quiet precordium, and no murmurs. Further investigations reveal a ventricular septal defect and Eisenmenger syndrome. The steps of clinical evaluation from history to examination and investigations are discussed. The management given and potential complications and their management are discussed.

Keywords: Cyanosis, eisenmenger syndrome, pulmonary hypertension


How to cite this article:
Ahmed J, Basha A, Pandey P, Parashar NK, Gupta S. Bedside case discussion. J Pract Cardiovasc Sci 2018;4:218-23

How to cite this URL:
Ahmed J, Basha A, Pandey P, Parashar NK, Gupta S. Bedside case discussion. J Pract Cardiovasc Sci [serial online] 2018 [cited 2019 Mar 19];4:218-23. Available from: http://www.j-pcs.org/text.asp?2018/4/3/218/249930


  Presenting Complaint Top


An 18-year-old boy, student of a secondary school, resident of Bihar, India, presented to us with:

  • Bluish discoloration of fingers since 5 years of age (13 years)
  • Dyspnea on exertion since 10 years of age (8 years).


The patient was apparently normal till 5 years of age when his parents started noticing bluish discoloration of his fingertips and toes. It was insidious in onset and gradually progressive in nature. He used to have a bluish discoloration of all the four limbs equalled. There was no history of any increase in cyanosis with excessively crying, exertion, and any episode of increase in cyanosis with labored breathing, fainting, or unconsciousness requiring medical help. The extent of cyanosis did not increase or decrease with changes in climatic temperature, any particular position such as squatting or any other maneuver. The parents also started noticing swelling of the fingertips and toes of all four limbs at about the same age. The swelling was also insidious in onset and gradually progressive in nature. After about 5 years of onset of cyanosis, he started having dyspnea on exertion which was insidious in onset and gradually progressive in nature, with a history of breathlessness on running and climbing two flights of stairs. He started taking medicine for the last 5 years and his dyspnea slightly improved so that he could climb up to three flights of stairs. There was no history of any orthopnea or paroxysmal nocturnal dyspnea, cough, expectoration, fever, seasonal variation in dyspnea severity, chest pain, palpitation swelling in feet, facial puffiness, or abdominal swelling.

There was no other significant history in the past. The child was born in a local hospital by normal vaginal delivery at term. There was a history of delayed cry after birth with meconium aspiration which resolved with treatment. There was neither history of any maternal complication nor any history of any maternal infection. There is no developmental delay in comparison to other siblings. There was no family history of any cardiac disease. The child was a product of nonconsanguineous marriage. He is second of four siblings and all siblings enjoying good health.


  Differential Diagnosis Based on History Top


This is a well-preserved, 18-year-old boy, known cyanotic with clubbing for 5 years of age.

The possibility is of a cyanotic heart disease, and the possibilities we would keep would include:

  1. Tetrology of Fallot
  2. Double outlet Right ventricle with Subaortic VSD with Pulmonary stenosis
  3. Left-to-right shunt (VSD, patent ductus arteriosus, or atrial septal defect [ASD]) with Eisenmenger syndrome (ES)
  4. ASD with PS
  5. Ebstein's anomaly.



  Examination Top


On examination, the height of the child was 155 cm, weight 35 kg, and body mass index 15.56 kg/m2. Blood pressure was normal with no significant difference in any of the limbs measuring 108/73 mmHg in right arm, SpO2 was 76% in all four limbs in room air, respiratory rate: 19/min, pulse: 80/min, regular, normal volume, all pulse palpable, jugular venous pulse: not elevated. Cyanosis was present with no significant difference in upper and lower limbs. Cyanosis was present with no significant differences in the UL and LL; clubbing Grade III was found in all four limbs. There was conjunctival plethora.

Cardiovascular examination revealed no precordial bulge, no visible pulsations over precordium or epigastric or suprasternal area, no scar marks, and no prominent veins over precordium. The apex was in the fifth intercostal space (ICS) 1-cm medial to medial collateral ligament, of normal character and localized, with a Grade I parasternal heave, no palpable heart sounds, no palpable P2, and no epigastric/liver pulsation.

On percussion, the right heart border corresponded to the right sternal margin, the left heart border corresponded to the apex, the left second prostate-specific antigen was resonant, and the upper border of liver dullness was in the right sixth ICS.

On auscultation, S1 was normal, and S2 was single and loud, with no additional sounds or murmurs.

The abdomen was soft and nontender; liver dullness was on the right side.

Neurological examination was unremarkable.


  Differential Diagnosis After Examination Top


Based on the above history and physical examination, a diagnosis of cyanotic congenital heart disease with decreased pulmonary blood flow was thought of.

  1. VSD Eisenmenger
  2. TOF.


Other possibilities

  • DORV with VSD with PS
  • Congenitally corrected transposition of the great arteries (CCTGA) with VSD with PS.


The outright presentation of this patient with cyanosis and dyspnea without any history of childhood respiratory tract infections and failure to thrive were pointers toward the cyanotic heart disease without decreased pulmonary blood flow. TOF usually comes to light in neonates and infants; however, when the shunt is balanced or left to right, the initial clinical course may be benign and patients may just have mild, intermittent, or stress-induced cyanosis. However, patients seldom remain acyanotic after thefirst few years of life, and by 5–8 years of age, most children are conspicuously cyanotic, with the cyanosis closely coupled to the severity of PS. No history of any cyanotic spells and late presentation with no PS murmur were against the clinical diagnosis of TOF.

The next clinical differential diagnosis in this patient was ES. Patients with ES often have a history of pulmonary congestion during infancy due to the left-to-right shunt with increased pulmonary blood flow. With increasing pulmonary vascular resistance, the pulmonary flow declines and pulmonary congestion decreases. The shunt will reverse and the patients become cyanotic with further increase in pulmonary vascular resistance. Typical clinical signs and symptoms of adults with ES are dyspnea on exertion, fatigue, syncope due to low systemic cardiac output, neurologic abnormalities (e.g., headache, dizziness, and visual disturbances) due to secondary erythrocytosis and hyperviscosity, congestive right heart failure, arrhythmias, hemoptysis due to pulmonary infarction, rupture of a dilated pulmonary artery or a thin-walled pulmonary arteriole, bleeding due to coagulation abnormalities or thrombocytopenia, and cerebrovascular accidents due to hyperviscosity, paradoxical embolism, or cerebral abscess. Physical examination reveals central cyanosis and clubbing. A parasternal lift from the right ventricle is palpable in almost all patients. The left ventricular apical impulse is usually absent due to a posterior displacement. At the upper left sternal border, a prominent main pulmonary artery impulse is present. In our patient, the pointers toward ES were late onset of symptoms, outright cyanosis and clubbing, physical findings of parasternal heave, single loud P2, and absence of any murmurs. Lack of any clinical features of any right-to-left shunt in infancy was odd points for ES; however, in developing countries, it is not uncommon to find children presenting for medical care after full-blown ES.

DORV with subaortic VSD with PS closely resembles TOF with similar clinical manifestations. PS, which varies from mild-to-severe to atresia, can be present at birth or delayed with a progressive increase in severity. The gentle right ventricular impulse, a systolic thrill felt maximally in the third ICS, an inconspicuous left ventricular impulse, pulmonary stenotic murmur which varies inversely with the severity of cyanosis are the typical findings quite resembling to TOF. When PS is mild, cyanosis coexists with increased pulmonary blood flow. When PS is severe, echocardiography (ECG) finding of counterclockwise initial vectors, namely q-waves in leads 1 and aVL despite a vertical or rightward QRS axis, is classical of DORV with subaortic VSD with PS. The PR interval tends to be prolonged. Terminal forces are slurred in leads 1, aVL, and avR and in leads V5-6. A decrescendo systolic murmur persists at the lower left sternal border because of obligatory flow from left ventricle across the VSD into the right ventricular aorta. In our patient, late presentation, absent pulmonary stenotic murmer, and lack of the classical ECG findings were highly unfavorable for the diagnosis of DORV with subaortic VSD with PS.

In CCTGA with VSD with PS, PS regulates the left-to-right shunt through a VSD. The pulmonary stenotic murmur is maximal at the midleft sternal border because the obstruction is subpulmonary, and the murmur radiates upward and to the right because of the rightward position and course of the pulmonary trunk. A loud second heart sound in the second left ICS is aortic because the aortic root is anterior. In the chest X-ray, the posterior pulmonary trunk is not border forming even if dilated and the ECG usually shows left-axis deviation, reverses septal depolarization with absence of q-waves in the left leads, and varying degrees of atrioventricular conduction abnormalities.

In our patient, lack of VSD murmur, single loud S2, and prominent pulmonary trunk in the chest X-ray [Figure 1] and ECG showing right-axis deviation were highly atypical for CCTGA with VSD with PS [Figure 2].
Figure 1: Chest X-ray revealing right ventricular type of apex, prominent pulmonary trunk, peripheral pulmonary pruning, and pulmonary oligemia.

Click here to view
Figure 2: Electrocardiography showing right-axis deviation, peaked P-waves, and Type III right ventricular hypertrophy.

Click here to view


With the above history and clinical examination the most likely diagnosis was Eisenmenger syndrome-VSD.

The patient underwent two-dimensional thoracic, which revealed dilated right atrium and right ventricle with subaortic nonrestrictive VSD with bidirectional shunting with severe pulmonary arterial hypertension (PAH) [Figure 3], [Figure 4], [Figure 5], [Figure 6].
Figure 3: Modified apical five-chamber view showing a large perimembranous ventricular septal defect (20 mm in size) marked by yellow arrow. RA: Right atrium, LA: Left atrium, LV: Left ventricle, RV: Right ventricle, IVS: Interventricular septum, VSD: Ventricular septal defect.

Click here to view
Figure 4: Parasternal long-axis view showing ventricular septal defect. (b) Peak gradient as measured with continuous-wave Doppler across the ventricular septal defect is 6 mmHg which is suggestive of nonrestrictive ventricular septal defect and severe pulmonary artery hypertension. LA: Left atrium.

Click here to view
Figure 5: Parasternal short-axis view at the level of aortic valve showing bidirectional flow through ventricular septal defect.

Click here to view
Figure 6: Parasternal short-axis view to demonstrate anatomy of pulmonary arteries. (a) Pulmonary annulus and arteries were of adequate size, (b) there was no pulmonic stenosis, (c) pulmonary velocity acceleration time of 77 ms suggests severe pulmonary arterial hypertension.

Click here to view


The patient was counseled about the course and prognosis of the disease, influenza, and pnemococcal vaccines were given and started on sildenafil 20 mg thrice a day as per the guideline recommendations and scientific data. It was planned to start diuretics if patient worsens symptomatically.

Despite absent scientific data and guideline recommendations, many ES patients are still treated with digoxin, standard heart failure medication, and subjected to oral anticoagulation or aspirin treatment. The use of digoxin is concerning as it has been demonstrated in other cardiovascular settings to increase mortality. Other standard heart failure drugs, namely angiotensin-converting enzyme inhibitors and angiotensin receptor blockers, may not share the problem of increasing mortality in their own right, but they have a hypotensive effect and may preclude the use of disease targeting therapy in adequate doses and especially combination therapy. Therefore, the use of these drugs should be very restrictive in the absence of guideline recommendations and positive scientific data. The issue of anticoagulation remains controversial. While a recent study found no evidence of a specific survival benefit in congenital pulmonary hypertension patients, current guidelines discourage the routine use of oral anticoagulation in ES patients in the absence of additional indications. Selected patients with large pulmonary thrombi may benefit from this therapy. It is likely, however, that a proportion of patients are given oral anticoagulation based on recommendations extrapolated from – but specifically intended for – patients with idiopathic pulmonary hypertension.

Other issues

The distinguishing features between different shunt lesions with pulmonary hypertension are shown in [Table 1], [Table 2], [Table 3] (adapted from the book “cardiology clinical methods by VJ Jose and S Ramakrishnan, Jaypee Brothers Medical Publishers;First edition 2017”).
Table 1: Clinical differences between Hyperkinetic and obstructive PAH

Click here to view
Table 2: Differentiating the types of Eisenmenger

Click here to view
Table 3: The differences between tetralogy of Fallot and Eisenmenger syndrome

Click here to view


Management of a patient with Eisenmenger syndrome

The management of the patient depends on the age, degree of cyanosis, and polycythemia. There is a risk of syncope, paradoxic embolism, hemoptysis, heart failure, and infective endocarditis. Dehydration should be avoided.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20]

  • Drugs such as diuretics and digoxin are used for heart failure when manifest
  • Oxygen therapy may be useful
  • Infective endocarditis prophylaxis should be carried out
  • Drugs commonly used include:[21],[22],[23],[24],[25],[26],[27],[28],[29],[30],[31],[32],[33],[34],[35],[36],[37],[38],[39],[40],[41]
    • Endothelin antagonists (bosentan, ambrisentan, and macitentan)
    • Phosphodiesterase inhibitors (sildenafil and tadalafil)
    • Others options such as prostaglandins can be tried.
  • Anticoagulation: not usually given
  • Contraception: Female patients
    • Fetal mortality 25%
    • Maternal mortality 50% (especially 1st postpartum week).
  • Erythrocytocis: symptoms of hyperviscousity with visual disturbances, fatigue, headache, dizziness, and paresthesia
    • Dehydration should be avoided
    • Phelebotomy should be done to keep Haematocrit of less than 65% in the presence of clinical symptoms of hyperviscosity syndrome.
  • Interventions
    • Creating an ASD with systemic or suprasystemic pressures
    • Lung or heart–lung transplant (lung transplant with repair of cardiac lesions in simple cardiac lesions else a heart–lung transplant):
    • Timing of transplant listing:
      • The New York Heart Association Functional Class III or IV despite a trial of at least
      • months of combination therapy including prostanoids
      • Cardiac index of <2 L/min/m2
      • Mean right atrial pressure of >15 mmHg
      • 6-min walk test of <350 m
      • Development of significant hemoptysis, pericardial effusion, or signs of progressive right heart failure (renal insufficiency, increasing bilirubin, brain natriuretic peptide, or recurrent ascites).



  Conclusion Top


This patient presented with cyanosis and a diagnosis of VSD Eisenmenger was made. The diagnostic steps at bedside and subsequent management issues are discussed.

Atfirst presentation, all patients should be classified as primary PAH or Eisenmenger or chronic thromboembolic pulmonary hypertension. The functional status should be classified with a 6-min walk test and the WHO functional class and whether the patient is in heart failure or not and what is the hemoglobin and saturation.

Patients with heart failure are given diuretics with or without digoxin.

Vasodilators are started when patients get symptomatic with shortness of breath with sildenafil (cheapest to start) or tadalafil (once daily, similar costs, less headache) alone or in combination with ambrisentan/bosentan (needs liver function monitoring)/macitentan (evidence base for prognostic advantage).

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Wood P. The eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J 1958;2:755-62.  Back to cited text no. 1
    
2.
Vongpatanasin W, Brickner ME, Hillis LD, Lange RA. The eisenmenger syndrome in adults. Ann Intern Med 1998;128:745-55.  Back to cited text no. 2
    
3.
Beghetti M, Galiè N. Eisenmenger syndrome a clinical perspective in a new therapeutic era of pulmonary arterial hypertension. J Am Coll Cardiol 2009;53:733-40.  Back to cited text no. 3
    
4.
Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004;351:1425-36.  Back to cited text no. 4
    
5.
Kidd L, Driscoll DJ, Gersony WM, Hayes CJ, Keane JF, O'Fallon WM, et al. Second natural history study of congenital heart defects. Results of treatment of patients with ventricular septal defects. Circulation 1993;87:I38-51.  Back to cited text no. 5
    
6.
Onat T, Ahunbay G, Batmaz G, Celebi A. The natural course of isolated ventricular septal defect during adolescence. Pediatr Cardiol 1998;19:230-4.  Back to cited text no. 6
    
7.
Saha A, Balakrishnan KG, Jaiswal PK, Venkitachalam CG, Tharakan J, Titus T, et al. Prognosis for patients with eisenmenger syndrome of various aetiology. Int J Cardiol 1994;45:199-207.  Back to cited text no. 7
    
8.
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;15:100-5.  Back to cited text no. 8
    
9.
Mebus S, Schulze-Neick I, Oechslin E, Niwa K, Trindade PT, Hager A, et al. The adult patient with eisenmenger syndrome: A medical update after dana point part II: Medical treatment – Study results. Curr Cardiol Rev 2010;6:356-62.  Back to cited text no. 9
    
10.
Diller GP, Alonso-Gonzalez R, Kempny A, Dimopoulos K, Inuzuka R, Giannakoulas G, et al. B-type natriuretic peptide concentrations in contemporary eisenmenger syndrome patients: Predictive value and response to disease targeting therapy. Heart 2012;98:736-42.  Back to cited text no. 10
    
11.
Diller GP, Kempny A, Inuzuka R, Radke R, Wort SJ, Baumgartner H, et al. Survival prospects of treatment naïve patients with eisenmenger: A systematic review of the literature and report of own experience. Heart 2014;100:1366-72.  Back to cited text no. 11
    
12.
Oechslin E, Mebus S, Schulze-Neick I, Niwa K, Trindade PT, Eicken A, et al. The adult patient with eisenmenger syndrome: A Medical update after dana point part III: Specific management and surgical aspects. Curr Cardiol Rev 2010;6:363-72.  Back to cited text no. 12
    
13.
Kaemmerer H, Mebus S, Schulze-Neick I, Eicken A, Trindade PT, Hager A, et al. The adult patient with eisenmenger syndrome: A medical update after dana point part I: Epidemiology, clinical aspects and diagnostic options. Curr Cardiol Rev 2010;6:343-55.  Back to cited text no. 13
    
14.
Maron BA, Galiè N. Diagnosis, treatment, and clinical management of pulmonary arterial hypertension in the contemporary era: A Review. JAMA Cardiol 2016;1:1056-65.  Back to cited text no. 14
    
15.
Matsubara H, Ogawa A. Treatment of idiopathic/hereditary pulmonary arterial hypertension. J Cardiol 2014;64:243-9.  Back to cited text no. 15
    
16.
Ueno T, Smith JA, Snell GI, Williams TJ, Kotsimbos TC, Rabinov M, et al. Bilateral sequential single lung transplantation for pulmonary hypertension and eisenmenger's syndrome. Ann Thorac Surg 2000;69:381-7.  Back to cited text no. 16
    
17.
Das BB, Wolfe RR, Chan KC, Larsen GL, Reeves JT, Ivy D, et al. High-altitude pulmonary edema in children with underlying cardiopulmonary disorders and pulmonary hypertension living at altitude. Arch Pediatr Adolesc Med 2004;158:1170-6.  Back to cited text no. 17
    
18.
Bowyer JJ, Busst CM, Denison DM, Shinebourne EA. Effect of long term oxygen treatment at home in children with pulmonary vascular disease. Br Heart J 1986;55:385-90.  Back to cited text no. 18
    
19.
Sandoval J, Aguirre JS, Pulido T, Martinez-Guerra ML, Santos E, Alvarado P, et al. Nocturnal oxygen therapy in patients with the eisenmenger syndrome. Am J Respir Crit Care Med 2001;164:1682-7.  Back to cited text no. 19
    
20.
Gonzaga LR, Matos-Garcia BC, Rocco IS, Begot I, Bolzan DW, Tatani SB, et al. Effects of acute oxygen supplementation on functional capacity and heart rate recovery in eisenmenger syndrome. Int J Cardiol 2017;231:110-4.  Back to cited text no. 20
    
21.
Lange RA, Brickner ME. Improving survival in patients with eisenmenger syndrome: Are we any closer? Circulation 2017;135:1441-3.  Back to cited text no. 21
    
22.
Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999;99:1858-65.  Back to cited text no. 22
    
23.
Fernandes SM, Newburger JW, Lang P, Pearson DD, Feinstein JA, Gauvreau K, et al. Usefulness of epoprostenol therapy in the severely ill adolescent/adult with eisenmenger physiology. Am J Cardiol 2003;91:632-5.  Back to cited text no. 23
    
24.
Ivy DD, Doran A, Claussen L, Bingaman D, Yetman A. Weaning and discontinuation of epoprostenol in children with idiopathic pulmonary arterial hypertension receiving concomitant bosentan. Am J Cardiol 2004;93:943-6.  Back to cited text no. 24
    
25.
El Yafawi R, Wirth JA. What is the role of oral prostacyclin pathway medications in pulmonary arterial hypertension management? Curr Hypertens Rep 2017;19:97.  Back to cited text no. 25
    
26.
Feldman J, Habib N, Radosevich J, Dutt M. Oral treprostinil in the treatment of pulmonary arterial hypertension. Expert Opin Pharmacother 2017;18:1661-7.  Back to cited text no. 26
    
27.
Maiya S, Hislop AA, Flynn Y, Haworth SG. Response to bosentan in children with pulmonary hypertension. Heart 2006;92:664-70.  Back to cited text no. 27
    
28.
Rosenzweig EB, Ivy DD, Widlitz A, Doran A, Claussen LR, Yung D, et al. Effects of long-term bosentan in children with pulmonary arterial hypertension. J Am Coll Cardiol 2005;46:697-704.  Back to cited text no. 28
    
29.
Kaya MG, Lam YY, Erer B, Ayhan S, Vatankulu MA, Nurkalem Z, et al. Long-term effect of bosentan therapy on cardiac function and symptomatic benefits in adult patients with eisenmenger syndrome. J Card Fail 2012;18:379-84.  Back to cited text no. 29
    
30.
Gatzoulis MA, Beghetti M, Galiè N, Granton J, Berger RM, Lauer A, et al. Longer-term bosentan therapy improves functional capacity in eisenmenger syndrome: Results of the BREATHE-5 open-label extension study. Int J Cardiol 2008;127:27-32.  Back to cited text no. 30
    
31.
Elshafay A, Truong DH, AboElnas MM, Idrees H, Metwali HG, Vuong NL, et al. The effect of endothelin receptor antagonists in patients with eisenmenger syndrome: A systematic review. Am J Cardiovasc Drugs 2018;18:93-102.  Back to cited text no. 31
    
32.
Ereminienė E, Kinderytė M, Miliauskas S. Impact of advanced medical therapy for the outcome of an adult patient with eisenmenger syndrome. Respir Med Case Rep 2017;21:16-20.  Back to cited text no. 32
    
33.
Humpl T, Reyes JT, Holtby H, Stephens D, Adatia I. Beneficial effect of oral sildenafil therapy on childhood pulmonary arterial hypertension: Twelve-month clinical trial of a single-drug, open-label, pilot study. Circulation 2005;111:3274-80.  Back to cited text no. 33
    
34.
Raja SG, Danton MD, MacArthur KJ, Pollock JC. Effects of escalating doses of sildenafil on hemodynamics and gas exchange in children with pulmonary hypertension and congenital cardiac defects. J Cardiothorac Vasc Anesth 2007;21:203-7.  Back to cited text no. 34
    
35.
Singh TP, Rohit M, Grover A, Malhotra S, Vijayvergiya R. A randomized, placebo-controlled, double-blind, crossover study to evaluate the efficacy of oral sildenafil therapy in severe pulmonary artery hypertension. Am Heart J 2006;151:851.e1-5.  Back to cited text no. 35
    
36.
Mukhopadhyay S, Sharma M, Ramakrishnan S, Yusuf J, Gupta MD, Bhamri N, et al. Phosphodiesterase-5 inhibitor in eisenmenger syndrome: A preliminary observational study. Circulation 2006;114:1807-10.  Back to cited text no. 36
    
37.
Ivy DD, Griebel JL, Kinsella JP, Abman SH. Acute hemodynamic effects of pulsed delivery of low flow nasal nitric oxide in children with pulmonary hypertension. J Pediatr 1998;133:453-6.  Back to cited text no. 37
    
38.
Nishimura RA, Carabello BA, Faxon DP, Freed MD, Lytle BW, O'Gara PT, et al. ACC/AHA 2008 guideline update on valvular heart disease: Focused update on infective endocarditis: A report of the American College of Cardiology/American Heart Association task force on practice guidelines: Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation 2008;118:887-96.  Back to cited text no. 38
    
39.
Linderkamp O, Klose HJ, Betke K, Brodherr-Heberlein S, Bühlmeyer K, Kelson S, et al. Increased blood viscosity in patients with cyanotic congenital heart disease and iron deficiency. J Pediatr 1979;95:567-9.  Back to cited text no. 39
    
40.
Gleicher N, Midwall J, Hochberger D, Jaffin H. Eisenmenger's syndrome and pregnancy. Obstet Gynecol Surv 1979;34:721-41.  Back to cited text no. 40
    
41.
Moceri P, Kempny A, Liodakis E, Alonso Gonzales R, Germanakis I, Diller GP, et al. Physiological differences between various types of eisenmenger syndrome and relation to outcome. Int J Cardiol 2015;179:455-60.  Back to cited text no. 41
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
 
 
    Tables

  [Table 1], [Table 2], [Table 3]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
   Abstract
  Presenting Complaint
   Differential Dia...
  Examination
   Differential Dia...
  Conclusion
   References
   Article Figures
   Article Tables

 Article Access Statistics
    Viewed228    
    Printed9    
    Emailed0    
    PDF Downloaded104    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]