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Eisenmenger Syndrome Clinical Presentation

  • Author: Mikhael F El-Chami, MD; Chief Editor: Park W Willis IV, MD  more...
 
Updated: Nov 23, 2014
 

History

Symptoms related specifically to pulmonary hypertension result from the inability to increase pulmonary blood flow in response to physiologic stress. Other symptoms are caused by various multisystem complications associated with cyanotic congenital heart disease. The Dana Point studies offer clinical aspects and diagnostic options,[19] medical treatments,[19] and surgical options.[20]

Symptoms of pulmonary hypertension include the following:

  • Breathlessness
  • Fatigue
  • Lethargy
  • Severely reduced exercise tolerance with a prolonged recovery phase
  • Presyncope
  • Syncope

Symptoms of heart failure include the following:

  • Exertional dyspnea
  • Orthopnea
  • Paroxysmal nocturnal dyspnea
  • Edema
  • Ascites
  • Anorexia
  • Nausea

Symptoms of erythrocytosis include the following:

  • Myalgias
  • Muscle weakness
  • Anorexia
  • Fatigue
  • Lassitude
  • Paresthesias of the digits and lips
  • Tinnitus
  • Blurred or double vision
  • Scotomata
  • Headache
  • Dizziness
  • Slowed mentation
  • Decreased alertness
  • Irritability

Symptoms of a tendency toward bleeding include the following:

  • Mild mucocutaneous bleeding
  • Epistaxis
  • Menorrhagia
  • Pulmonary hemorrhage

Symptoms of vasodilation include the following:

  • Presyncope
  • Syncope

Symptoms of cholelithiasis include the following:

  • Right upper quadrant pain
  • Biliary colic
  • Fever
  • Pale stools
  • Jaundice

Symptoms of nephrolithiasis include the following:

  • Renal colic
  • Secondary gout
  • Joint pain and swelling

Additional symptoms include the following:

  • Paradoxical embolus can cause symptoms of localized vascular insufficiency end-organ ischemia
  • Hypertrophic osteoarthropathy can cause long bone pain and tenderness
  • Retinal complications include episodes of transient visual loss and spontaneous hyphemas

Patients who develop Eisenmenger syndrome may be asymptomatic for long periods of time. The elevated pulmonary vascular resistance (PVR) prevents pulmonary overcirculation and the symptoms of heart failure. This can result in a delay in diagnosis.

In the first weeks of life when the PVR begins to fall toward adult levels, an infant with a large atrial septal defect (ASD), ventricular septal defect (VSD), or patent ductus arteriosus (PDA) may present with congestive heart failure symptoms due to the large left-to-right shunt. This may simply be reflected by poor weight gain.

Infants with the same defects who maintain a high PVR have less left-to-right intracardiac shunting and less pulmonary blood flow. Therefore, developing Eisenmenger physiology may remain undetected in infants with a high PVR and relatively large defects because they lack a loud systolic murmur and/or diastolic rumble and the symptoms of heart failure.

Patients may have a period of poor weight gain, reflecting congestive heart failure, that improves as pulmonary pressures increase and overcirculation decreases. Clues to the diagnosis may include only dyspnea upon exertion and exercise intolerance. These symptoms become increasingly evident with advancing age, particularly at adolescence, and may progress to lethargy and syncopal episodes.

Erythrocytosis secondary to chronic cyanosis is an adaptation to low levels of circulating oxyhemoglobin and is present in most patients. Excessive polycythemia may result in hematocrit levels of greater than 65% and hyperviscosity syndrome. Hyperviscosity may lead to thromboembolic events, cerebrovascular complications, gout, chest pain from pulmonary infarction, and hemoptysis. Most of the symptoms are nonspecific and are confirmed if they are relieved by phlebotomy.

Any of the multitude of multisystem complications that occur in patients with congenital heart disease may be present.

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Physical Examination

Cardiovascular findings include the following:

  • Central cyanosis (differential cyanosis in the case of a PDA)
  • Clubbing
  • Jugular venous pulse wave may be A-wave dominant, and, in the presence of a significant tricuspid regurgitation, the V wave may be prominent; central venous pressure may be elevated.
  • Precordial palpation reveals a right ventricular heave and, frequently, a palpable S 2.
  • Loud P 2
  • High-pitched early diastolic murmur of pulmonic insufficiency
  • Right-sided fourth heart sound
  • Pulmonary ejection click
  • Single S 2

As the pulmonary vascular resistance progressively rises, the holosystolic murmur of a nonrestrictive VSD shortens and softens, first becoming early systolic in timing, before disappearing entirely as the shunt is reversed.

The continuous murmur of a PDA disappears when Eisenmenger physiology develops; a short systolic murmur may remain audible.

Other signs of Eisenmenger syndrome include the following:

  • Respiratory signs - Include cyanosis and tachypnea
  • Hematologic signs - Include bruising and bleeding; funduscopic abnormalities related to erythrocytosis include engorged vessels, papilledema, microaneurysms, and blot hemorrhages
  • Abdominal signs - Include jaundice, right upper quadrant tenderness, and positive Murphy sign (acute cholecystitis)
  • Vascular signs - Include postural hypotension and focal ischemia (paradoxical embolus)
  • Musculoskeletal signs - Include clubbing, tenderness over the metacarpal or metatarsal joints (hypertrophic osteoarthropathy), and joint effusions
  • Cutaneous signs - The skin demonstrates fewer urate deposits than are commonly observed in primary gout
  • Ocular signs - Include conjunctival injection, rubeosis iridis, and retinal hyperviscosity changes

Clinical findings in progressing disease

Examination findings vary with the progression of the disease. Early in life, infants with a large systemic-to-pulmonary communication may demonstrate mild pulmonary overcirculation with symptoms of cor pulmonale. Initially, cyanosis is absent, and infants present with the signs and symptoms of heart failure. Physical examination may reveal tachypnea, nasal flaring, grunting, retractions, and tachycardia.

An auscultatory examination may reveal a hyperactive precordium, systolic flow murmur, diastolic rumble, and hepatosplenomegaly. Cardiac examination findings are determined by the underlying anatomic defects. Delayed capillary refill may be present, indicating low cardiac output.

As the PVR increases, the pulmonary circulation receives less blood flow, with gradually advancing pulmonary artery pressure. Symptoms of congestive heart failure wane. The right ventricle may become hypertrophied, and the chest, when examined, may be asymmetrical, with a right ventricular heave and a palpable P2 (ie, a pulmonary closure sound that is so forceful that it can be felt).

As pulmonary resistance increases over time, a relative decrease in the left-to-right intracardiac shunting occurs, initially with periods of subclinical right-to-left and bidirectional shunting, followed by frank cyanosis, clubbing, and polycythemia (giving a ruddy appearance to the skin).

A hallmark of Eisenmenger syndrome is this seemingly improved clinical condition, despite the lack of change in therapy for congestive heart failure. It represents a physiologically normalized condition caused by the progressively worsening pulmonary vascular obstructive disease (PVOD), with resolution of pulmonary overcirculation and heart failure.

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Contributor Information and Disclosures
Author

Mikhael F El-Chami, MD Assistant Professor, Department of Cardiology, Division of Electrophysiology, Emory University School of Medicine

Mikhael F El-Chami, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, Heart Rhythm Society

Disclosure: Received grant/research funds from Medtronic Inc for principle investigator.

Coauthor(s)

Charles D Searles, Jr, MD Assistant Professor of Medicine, Division of Cardiology, Emory University School of Medicine; Consulting Staff, Division of Cardiology, Director of Stress Echo Laboratory, Grady Memorial Hospital

Charles D Searles, Jr, MD is a member of the following medical societies: American Heart Association, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Park W Willis IV, MD Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

Acknowledgements

Stuart Berger, MD Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose

Brian M Cummings, MD Pediatric Critical Care; Director Pediatric Transport, Medical Director PALS, MassGeneral Hospital for Children, Instructor in Pediatrics, Harvard Medical School

Brian M Cummings, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Elyse Foster, MD Director of Adult Echocardiography Laboratory and Adult Congenital Heart Disease Service, Department of Internal Medicine, Division of Cardiology, Moffitt Hospital; Assistant Professor of Cardiology, University of California, San Francisco, School of Medicine

Elyse Foster, MD is a member of the following medical societies American College of Cardiology, American College of Physicians, American Heart Association, and American Society of Echocardiography

Disclosure: Nothing to disclose.

Lisa A Hourigan, MBBS, FRACP Consulting Staff, Department of Cardiology, University of California, San Francisco School of Medicine

Disclosure: Nothing to disclose.

Christopher Johnsrude, MD, MS Chief, Division of Pediatric Cardiology, University of Louisville School of Medicine; Director, Congenital Heart Center, Kosair Children's Hospital

Christopher Johnsrude, MD, MS is a member of the following medical societies: American Academy of Pediatrics and American College of Cardiology

Disclosure: St Jude Medical Honoraria Speaking and teaching

John W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatric Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital

John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Jeff L Myers, MD, PhD Chief, Pediatric and Congenital Cardiac Surgery, Department of Surgery, Massachusetts General Hospital; Associate Professor of Surgery, Harvard Medical School

Jeff L Myers, MD, PhD is a member of the following medical societies: American College of Surgeons, American Heart Association, and International Society for Heart and Lung Transplantation

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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This radiograph reveals an enlarged right heart and pulmonary artery dilatation in a 24-year-old woman with an unrestricted patent ductus arteriosus (PDA) and Eisenmenger syndrome.
Apical, 4-chamber, transthoracic view demonstrating an ostium primum atrial septal defect (ASD) with enlarged right-side chambers. RA = right atrium, RV = right ventricle, LA = left atrium, LV = left ventricle.
This computed tomography (CT) chest scan shows a large, unrestricted patent ductus arteriosus (PDA) in a 24-year-old woman with Eisenmenger syndrome.
This apical, 4-chamber, transthoracic segment shows color Doppler flow across the interatrial septum at the site of a large ostium primum atrial septal defect (ASD). RA = right atrium, LA = left atrium.
This transesophageal image is from the midesophagus of a patient with Eisenmenger syndrome secondary to an unrestricted patent ductus arteriosus (PDA). It shows a severely dilated pulmonary artery. PA = pulmonary artery, Asc Ao = ascending aorta.
This is a color Doppler interrogation of the tricuspid valve in a patient with Eisenmenger syndrome. It demonstrates an elevated estimated right ventricular systolic pressure of 106 mm Hg + right atrial pressure, reflecting pulmonary hypertension. TR = tricuspid regurgitation.
This is the transthoracic Doppler examination of the pulmonic valve in a 24-year-old woman with Eisenmenger syndrome secondary to an uncorrected ostium primum atrial septal defect (ASD). This reveals an elevated estimated pulmonary artery diastolic pressure of 51 mm Hg + right atrial pressure. PR = pulmonic regurgitation.
 
 
 
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