Cardiomyopathies come from many mechanisms, but the conditions may be divided into three major types: First, dilated or congestive. Second, hypertrophic, and third, restrictive. A careful history-taking by the registered nurse or practitioner along with a complete physical examination can reveal cardiomyopathies, but it is appropriate to confirm the diagnosis with a transthoracic echocardiography and selected laboratory studies.
Key Points –
The hallmark of hypertrophic cardiomyopathy is myocardial hypertrophy (e.g., the myocardium thickening of the wall size and shape), that is inappropriate and often A-symmetric that occurs in the absence of an obvious inciting hypertrophy stimulus. Although any region of the left ventricle (LV) can be affected, hypertrophy frequently involves the interventricular septum which can result in an outflow tract obstruction. In many clinical studies regarding the electrophysiology of hypertrophic cardiomyopathy, patients have preserved systolic function with impaired LV compliance that results in diastolic dysfunction whether or not the outflow tract obstruction is present. 1
Clinical Manifestations –
The nurse- clinician should take extra steps in gathering patient and family history as in asking questions regarding some basic symptoms: Is there a history of a productive cough and dyspnea, perhaps with wheezing. Breathlessness limits the patient’s ability in the minor stresses of daily living. Frequently there is a history of emergency hospital admissions because of respiratory infection, and sometimes necessitating mechanical ventilation. In breathing oxygen (O₂), there may be increasing somnolence or other symptoms of hypercapnia such as recurring headaches, confusion, and even vomiting which when combined with blurred optic discs (also, due to cerebral vasodilation), constitutes the “pseudo tumor cerebri” syndrome. 2ʹ3
Note: Hypoxia is usually present due to hypoventilation and sleep- apnea that may worsen at night. Therefore, it may be wise to have a sleep study done to rule- out any pulmonary disease, along with labs for hemodynamics in patients with questionable obstructive pulmonary disease or episodes of peripheral edema on site. 4
Also, in regards to older adults, during the examination the nurse and/or clinician should look for nicotine staining of the fingers, which is a sign reflecting many years of heavy cigarette smoking. The skin may be warm and the arterial pulse bounding in the high cardiac output state induced by hypoxia and hypercapnia. The distention of the chest due to the airflow obstruction and the presence of rhonchi and wheezes secondary to chronic bronchitis usually make cardiac auscultation difficult. Take notice with your cardiac stethoscope of a right-sided protodiastolic galloping sound (S₃) and a systolic murmur of the tricuspid regurgitant which may be audible.
Note: As a registered nurse or treating practitioner, if you are given the opportunity to be with your patient while the service of a 2-D Echo is being performed, please ask the clinician while he or she is recording in the short axis mode, to listen and investigate heart sounds, by placing your cardiac stethoscope over the apex and moving it over the chambers to hear the various sounds. This will bring alive your sense of sight and sounds to a new level.
Signs of enlargement of the right ventricle (RV) may be attributed to mild-to-moderate pulmonary hypertension (PHTN) that is common in severe obstructive bronchitis and emphysema. Pulmonary artery systolic pressure is typically in the range of 50 to 60 mmHg, far below the systemic levels that may occur in patients with congenital heart disease and in those with primary pulmonary hypertension. 5
Note: The investigating registered nurse should be aware that patients with cor pulmonale due to “chronic obstructive lung disease” (COLD), usually have an advanced form of the disease with FEV₁ < 1.0 L and Pа₀₂ < 60 mmHg. Also, the RV failure secondary to COLD often occurs when there is “acute-on-chronic” respiratory failure with evidence of hypoxemia.6
Laboratory Examinations for Evidence Based -
The electrocardiogram (ECG) will often show sinus tachycardia (Sin tach) or atrial fibrillation (AFib), ventricular arrhythmias, and left atrial abnormalities. Also, look for diffuse non-specific ST-T wave abnormalities and sometimes an intraventricular conduction defect (IVCD), with global low voltage criteria. These are all electrical nodal signs of possible pre-cardiomyopathy. Next, order a complete 2-D echocardiography and radionuclide- ventriculography, which my show left ventricular dilatation, with normal or minimally thickened or thinned walls, along with systolic dysfunction (reduced ejection fraction).
Note: The nurse-clinician should investigate, identify, and document, then forward these signs and symptoms to the primary physician, in order for possible further investigation into ordering a cardiac catheterization and/or coronary angiography. In many case studies reported within the U.S. patients who pursue an inexorably down-hill course, and those who are over 55 years of age, die within 3 years of the onset of symptoms. 7
History Taking Protocols –
Patients with hypertrophic cardiomyopathy (HCM) may indeed appear A-symptomatic. The nurse and/or practitioner should be able to detect and document some of the signs and symptoms which can include dyspnea, syncope, pre-syncope, angina, palpitations, orthopnea, paroxysmal nocturnal dyspnea, dizziness, congestive heart failure, and through atopy findings, in sudden cardiac death.
Note: After your findings and documentation as an investigating nurse- clinician, consult with the cardiologist and/or electro-physiologist for possible specific cardiac studies which should include the non-invasive cardio-diagnostic department, to run either a Frank- vector-cardiogram or Single-Average (e.g., late potential diagnostics). Like syncope, pre-syncopal episodes warrant a direct evaluation to rule-out (R/O) malignant arrhythmias. A thorough investigation is warranted to also, R/O potential malignant etiology of pre-syncopal symptoms.10ʹ11
Note: All nursing-clinicians should keep in mind, that non-sustained ventricular tachycardia is another bio-marker, and is included for higher risk factors of sudden death.14
Orthopnea and Paroxysmal Nocturnal Dyspnea -
Note: The nurse’s sense (awareness) of importance due to acute findings through observation and documentations may allow her and/or him to ask the treating physician (based on specific evidence through critical findings) to order an electroencephalogram (EEG) study, or a computed tomography (CT) to R/O secondary neurological problems caused by primary HCM.
Congestive Heart Failure –
Note: The pathophysiology of CHF is similar in younger children, about 10% at initial presentation, most commonly in infants younger than 1 year of age.17 The systolic function in children with HCM is almost always well preserved, at least until the last stage of the disease. Patients with CHF have a high likelihood of recurrent heart failure due to both mitral regurgitation and profound diastolic dysfunction.18
Sudden Cardiac Death -
Physical Examination Findings –
Most patients with HCM or cardiomyopathy (CM), excluding the pediatric patients with known congenital heart disease, do not have outflow tract obstruction and may show completely normal during the physical examination findings. Therefore, it is important that the nurse investigates further, using her skills in evidence-based practice towards listening to heart sounds, cardiac impulse, murmur, and other findings:
Cardiac Impulse –
The hallmark of the restrictive cardiomyopathies is abnormal diastolic function; while the ventricular walls are excessively rigid and impede ventricular filing. Also, myocardial fibrosis, hypertrophy, or infiltration due to a variety of causes is usually responsible. The cardiologist and/or electro physiologist can determine through a series of diagnostic exams which may include the specific roentgen (chest x-rays), nuclear imaging, and a transesophageal -echocardiogram.
Note: The Transesophageal-Echocardiogram (ETT) procedure allows the cardiologist to view the heart while it is moving and to observe its main pumping chambers, along with the shape and thickness of the chamber walls as well. Through this unique procedure, it is also possible to determine the volume and direction of the blood flow cycle in the heart. This assessment is especially important for the HCM patient to determine size, pumping strength, abnormal blood flow patterns, and structural abnormalities.
The myocardial involvement with the protein “amyloid,” once deposited in the heart muscle, kidneys, or nervous system, it cannot be broken-down, nor recycled by the body and will lead to amyloidosis. The signs and/or symptoms that you may be observing will include: swollen legs and ankles, weakness, weight loss, shortness of breath, dizziness, diarrhea, severe fatigue, enlarge tongue, and numbness in the extremities. Also, this is a common cause of secondary restrictive cardiomyopathy. 28
In many of these conditions, particularly those with substantial concomitant endocardial involvement, partial obliteration of the ventricular cavity by fibrous tissue and thrombus contributes to the abnormally increased resistance to the ventricular filling. Thromboembolic complications have been reported in the U.S. in about a third of these patients. 29
HCM may be characterized morphologically and defined by a hypertrophied, non-dilated LV in the absence of another systemic or cardiac disease that is capable of producing an abnormal wall thickening evident (e.g., systemic hypertension and/or valvular stenosis). Indeed, clinical diagnosis for the most part and cost saving measures remain today through an electrocardiograph (12-lead ECG) or an event recorder (AECG). Plus a transthoracic 2-dimensional echocardiograph, and with a cardiac magnetic resonance imaging, by detection of otherwise unexplained abnormal LV function. Also including evidenced-based suspicion that may be raised after clinical profiling and family screening is gathered.
Medical research institution across the world has open new pathways into genetic encoding, and ion channelopathies, both nocturnal and sudden unexplained death syndromes in pediatric and older adults, are investigated through mutations in defective ionic channel proteins and ion channel electrical disorders which include the LQTS, and/or short-QT syndrome. Also notable, are the SQTS syndrome, and the Brugada syndrome which includes gene encoding. These electro-physiological findings are making there way into the care of the trauma physician teams within North American and Southeast Asia.
Throughout this article, the author’s have strived to bring a useful guideline towards the registered nurses and/or nursing practitioners, in order to enhance and encourage advance critical-thinking that she and/or he may act upon within a medical institution, outpatient walk-in clinic, or emergency room department.
Also, the authors would like to give their thanks to both learning institutions (Angeles College of Nursing and the advanced Continuing Nursing Education Department at Kaiser Permanente Health-Care Facilities for the advancement of applied nursing education and information. Also, the physicians (e.g., interns, 3rd year residence, and fellows), who are finishing their cardiology and electrophysiology services.
 Jarcho, J.A., McKenna, W., Pare, J.A., et al. Mapping a gene for familial hypertrophic cardiomyopathy to chromosome 14q1. N Engl J Med. Nov. 16, 1998; 321 (20): 1372-8.
 Incalzi, R.A., et al: Electrocardiographic signs of cor pulmonale: A negative prognostic finding in chronic obstructive pulmonary disease. Circulation 99: 1600, 1999.
 Weitzenblum, E., et al: Benefit from long-term O₂ therapy in chronic obstructive pulmonary disease patients. Respiration. 59 (Suppl. I): 14, 1994.
 Haraldsson, A. et al: Comparison of inhaled nitric oxide and inhaled aerosolized pros-tacylin in the evaluation of heart transplant candidates with elevated pulmonary vascular resistance. Chest 114:780, 1998.
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 Rich, S: Cor pulmonale, in: Heart Disease, 6th ed. E. Braunwald (ed). Philadelphia, Saunders, 2001.
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 Colan, S.D., Lipshultz, S.E., Lowe, A.M., et al. Epidemiology and cause-specific outcome of hypertrophic cardiomyopathy in children: findings from the Pediatric Cardiomyopathy Registry. Circulation. Feb. 13, 2007; 115(6): 773-81.
 Braunwald, E., (Sir. Ed). et al. 5th ed. Heart Disease: A Textbook of Cardiovascular Medicine. Vol. 1, 1997 edition. Symptoms of Heart Failure, Respiratory Distress: Dyspnea. Philadelphia: W.B. Saunders Co. pp. 439-43.
 Perloff, J.K., Middlekauff, H., Child, J.C., Goldberg, G.D., et al. Electrophysiologic Properties of the Atrialized Right Ventricle in Ebstein’s Anomaly. Published through the Ahmanson/UCLA Adult Congenital Heart Center and the Dept. of Pathology Laboratory of Medicine, and the David Geffen School of Medicine at UCLA, Los Angeles, Cal. Spring, 2005. Also, cited in Elsevier: Amer J Card (2005): Issue 12, under Science Direct Abstract.
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 Maron, B.J., Roberts, W.C., Epstein, S.E. Sudden death in hypertrophic cardiomyopathy: a profile of 78 patients. Circulation. Jun 1982: 65(7): 1388-94. Lippincott Williams & Wilkins Publishes the AHA Journals for the Amer H Assoc cited in: http://circ.ahajournals.org/
 Wolf, C.M., Moskowitz, I.P., Arno, S, et al. Somatic events modify hypertrophic cardiomyopathy pathology and link hypertrophy to arrhythmia. Proc Natl Acad Sci U S A. Dec. 13, 2005: 102(50):18123-8.
 Rich, M.W. et al. The Geriatric Cardiology Section: Barnes-Jewish Hospital, Washington Univ. Med. Center: St. Louis, Mo. USA. The Epidemiology, Pathophysiology, and Etiology of Congestive Heart Failure. J Amer Geriatr Soc. 1999, Apr. 46(4): 536-9.
 Morita, H, Rehm, H.L., Menesses, A. et al. Shared Genetic Causes of Cardiac Hypertrophy in Children and Adults. N Engl J Med. May 1, 2008; 358(18): 1899-908. http:/www.nejm.org// The New England Journal of Medicine is owned, published, and copyrighted © 2009 Massachusetts Medical Society.
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Short Bio of Dr. Gary D. Goldberg, PhD
Over 30 years experience in the Medical field, At UCLA and Pacific Hospital of the Valley, as a Chief Technologist and Analyst, Visiting Professor and Instructor for continuing education at UCLA School of Nursing and Writer/Speaker at the School of Medicine from 1995-2008.
From 2003-2008 Dr. Goldberg has collaborated and published through Blackwell Publishing Co. (Medical Division), and Journal of American College of Cardiology plus 15, published abstracts through UCLA Dept. of Internal Medicine and the Dept. of Cardiology.
Current title: Clinical Professor of Medical Education with Angeles College of Nursing, in Los Angeles, Ca.
Dr. Goldberg has written two major academic course textbooks for Angele College of Nursing and has represented advanced nursing education course curriculum through the State Dept. of California and approved by the ANA for CEU(s) and the AMA CMU Level 1 Credit for physicians.
His wife, Cindy L. Capute-Goldberg, has been a registered nurse for over 17 years and has managed a 200-bed acute care facility with over 100, professional nurses from RN’s through CNA in the Los Angeles area. She has co-authored with Dr. Goldberg in 2005, a manuscript presented to the Cardiology-Electrophysiology Research Group (i.e., DMPG), that has changed the dynamics of electro-static reading with regards to acute atrial anomalies.
This finding allowed the author to publish the ‘Goldberg Protocol’ for Cardiac placements in the field and under the Dept. of Medicine Chair, in using a tilt-table with the 12 +3 Leads or the vector positioning for additional cardiac patient information.
To reach Dr. Gary D. Goldberg, for comments and/or consultation, please use e-mail address: [email protected] or cell phone contact: 818-610-9017.