May 2024 Medical Image of the Month: Hereditary Hemorrhagic Telangiectasia in a Patient on Veno-Arterial Extra-Corporeal Membrane Oxygenation

Figure 1.  Preoperative nasopharyngoscopic direct visualization of telangiectasia of the nasal turbinate.

Figure 2.  Noncontrast head CT on postoperative day 3 demonstrates extensive multifocal areas of low attenuation consistent with early signs of infarction involving much of the cerebral hemispheres, most prominently involving the left parietal lobe.

A 54-year-old man with a complex cardiac history, including Tetralogy of Fallot requiring Blalock-Taussig shunt in infancy, infundibular patch repair at age 7, and bioprosthetic tricuspid valve replacement at age 52, had ongoing frequent hospitalizations with decompensated right ventricular heart failure secondary to native pulmonary valve mixed stenosis plus regurgitation and left pulmonary artery stenosis. His case was further complicated by his history of hereditary hemorrhagic telangiectasia (HHT) with recurrent epistaxis and recent GI bleeds with multiple angiodysplastic lesions throughout the stomach, duodenum, and descending colon which were previously treated with argon plasma coagulation.

The patient was admitted to our hospital in NYHA class IV heart failure receiving a continuous dopamine infusion and aggressive diuresis. Upon admission, a right heart catheterization demonstrated severe pulmonary valve regurgitation, left pulmonary artery stenosis, and systemic hypoxemia suggestive of an intrapulmonary shunt. Admission transthoracic echocardiogram demonstrated normal left ventricular ejection fraction of 55-60%, a severely enlarged right ventricle, moderately reduced right ventricular systolic function, severe pulmonary valve regurgitation, and moderate pulmonary valve stenosis.

A multidisciplinary team including congenital cardiology, pulmonary hypertension, interventional pediatric cardiology, and congenital cardiovascular surgery was consulted and after extensive discussions the patient consented to surgical intervention. Prior to his operative date, he underwent cauterization of his bilateral nasal cavity telangiectasias by Otolaryngology (Figure 1). On hospital day sixteen, he underwent a fourth time redo median sternotomy, pulmonary valve replacement with St. Jude Epic 27-mm porcine bioprosthesis, and repair of left pulmonary artery stenosis. Intraoperative transesophageal echocardiogram at the end of the surgical case demonstrated severe right ventricular dilation, severe right ventricular systolic dysfunction, normal pulmonary valve prosthesis, and left ventricular ejection fraction of 55%. The case was technically challenging requiring a cardiopulmonary bypass time of 178 minutes, and massive transfusion (including twelve units packed red blood cells, two packs of platelets, 4 units fresh frozen plasma, and 10 units cryoprecipitate) for a total estimated blood loss of 3.9 L.

Postoperatively, he had persistent right ventricular systolic dysfunction and diffuse mediastinal hemorrhage. By postoperative day two, a repeat transesophageal echocardiogram revealed worsening right ventricular dilation and severe right ventricle systolic dysfunction. The multidisciplinary care team recommended central venoarterial extracorporeal membrane oxygenation (VA ECMO) support for both worsening hypoxemia and continuing severe right ventricular failure.  The aorta was cannulated with a 22 French Bio-Medicus cannula (Medtronic, Minneapolis, USA) and the right atrium cannulated with a 36 French venous cannula (Medtronic, Minneapolis, USA), and full ECMO support was initiated using a Cardiohelp console with a HLS 7.0 oxygenator (Getinge, Goteborg, Sweden) reaching ECMO blood flows of 6 L/minute (an indexed ECMO blood flow of 2.6 L/minute/m²).

On POD 3, bronchoscopy was performed and revealed diffuse thin bloody secretions in the distal airways without a focal source, which was cleared with suction but quickly reaccumulated. Due to the pulmonary hemorrhage and recent mediastinal hemorrhage, systemic anticoagulation was not started at that time. Due to a lack of awakening during a sedation vacation, computed tomography (CT) imaging of his head was obtained and demonstrated a large ischemic stroke affecting the majority of the left MCA territory and part of the right parietal lobe (Figure 2).

HHT (also known as Osler-Weber-Rendu disease) is an autosomal dominant genetic disease with various vascular manifestations (1). In addition to the more common mucocutaneous and gastrointestinal tract telangiectasias, some patients with HHT also have pulmonary arteriovenous malformations (AVMs) with right-to-left shunt that can cause hypoxemia with resultant polycythemia. Cerebral AVMs present a risk of intracranial hemorrhage, ischemia, and hydrocephalus, which correlate with the size of the vascular defect. Given the presence of AVMs and hemorrhagic complications related to telangiectasias, the use of extracorporeal membrane oxygenation (ECMO) in patients with HHT is a potentially high-risk situation.

This case highlights the risks of ECMO in patients with HHT. The causes of this patient’s hemorrhagic and thrombotic events were most likely multifactorial, including contributions from a dilutional and consumptive coagulopathy after cardiopulmonary bypass and hemorrhage, initiation of ECMO, kidney failure, and his underlying HHT. The timing and precise cause of our patient’s cerebral infarction are unclear. However, patients with HHT and clinically significant intrapulmonary AVMs may have an increased risk of paradoxical thromboembolic stroke (2). The international HHT expert guidelines assert that even though HHT is a hemorrhagic disorder, it provides no protection against thrombosis (3). In addition, patients with HHT may  levels of von Willebrand factor and factor VIII, which would potentially increase their risk of thrombosis (4). This case exemplifies the substantial risks of hemorrhagic and thrombotic complications associated with ECMO for patients with HHT. Further study is needed to help determine whether HHT should be considered a contraindication to ECMO.

Theodore O. Loftsgard, APRN, CNP^1,2; Kari A. Wilson, APRN, CNP^1,2; John K. Bohman, MD^2,3

¹Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN

²Critical Care Independent Multidisciplinary Program, Mayo Clinic, Rochester, MN

³Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, Rochester, MN

References

1. Faughnan ME, Palda VA, Garcia-Tsao G, et al. International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J Med Genet. 2011 Feb;48(2):73-87. [CrossRef] [PubMed]
2. Dittus C, Streiff M, Ansell J. Bleeding and clotting in hereditary hemorrhagic telangiectasia. World J Clin Cases. 2015 Apr 16;3(4):330-7. [CrossRef] [PubMed]
3. Faughnan ME, Mager JJ, Hetts SW, et al. Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia. Ann Intern Med. 2020 Dec 15;173(12):989-1001. [CrossRef] [PubMed]
4. Shovlin CL, Sulaiman NL, Govani FS, Jackson JE, Begbie ME. Elevated factor VIII in hereditary haemorrhagic telangiectasia (HHT): association with venous thromboembolism. Thromb Haemost. 2007 Nov;98(5):1031-9. [PubMed]

Cite as: Loftsgard TO, Wilson KA, Bohman JK. May 2024 Medical Image of the Month: Hereditary Hemorrhagic Telangiectasia in a Patient on Veno-Arterial Extra-Corporeal Membrane Oxygenation. Southwest J Pulm Crit Care Sleep. 2024;28:68-70. doi: https://doi.org/10.13175/swjpccs015-24 PDF

Medical Image of the Month: Massive Right Atrial Dilation After Mitral Valve Replacement

Figure 1. Chest radiograph demonstrating massive cardiomegaly with pulmonary congestion and markedly dilated right atrium.

Figure 2. Transthoracic echocardiogram demonstrating marked dilation of the right atrium to 9.6 cm in its greatest dimension.

A 92-year-old woman with a history of mechanical mitral valve replacement (+25 years prior to presentation), coronary artery bypass grafting, pacemaker placement and heart failure (EF 25%) presented from a nursing facility for dyspnea of 1 day’s duration. Recently, the patient had experienced a bowel perforation s/p surgical repair 3 weeks prior.

Admission chest radiograph was significant for massive cardiomegaly with pulmonary congestion and markedly dilated right atrium (Figure 1). Formal echocardiography was ordered, which re-demonstrated the patient’s known heart failure with reduced ejection fraction. Additionally, all 4 chambers of the heart were noted to be dilated, but the right atrium was revealed to be severely enlarged to >9 cm (Figure 2). On review of outside records, the patient’s cardiac history was notable for chronic dilation of the RA, RV and LA for several years with low, but stable, LV ejection fraction. Ultimately, the patient was noted to have worsening abdominal distension concerning for acute abdomen. However rather than pursue additional aggressive work up after her recent surgery, comfort measures were preferred.

This case illustrates some of the possible long-term effects of mitral valve replacement. In recent years mitral valve repair has become the preferred method over replacement for degenerative valve disease in western countries (1). While there are documented short term benefits to both mitral valve replacement and mitral valve repair long term data is less available (2). Long-term survival in most studies is reported in 5,10, and 15-year intervals. As was the case with our patient, patients with mitral valve replacement greater than 20 years in age have significantly less information associated with them. Although at this time longitudinal studies suggest benefits for both mitral valve replacement and repair, further investigation into long term complications is warranted (3). As our society continues to age, understanding the risks and complications associated with previous valve repair will help guide therapeutic interventions in the geriatric patient.

Richard Young, MD* and Alexander Ravajy, BS**

*University of Arizona Department of Internal Medicine

**University of Oklahoma Department of Microbiology

Banner University Medical Center

Tucson, AZ USA

References

1. Gammie JS, Sheng S, Griffith BP, Peterson ED, Rankin JS, O'Brien SM, Brown JM. Trends in mitral valve surgery in the United States: results from the Society of Thoracic Surgeons Adult Cardiac Surgery Database. Ann Thorac Surg. 2009 May;87(5):1431-7. [CrossRef] [PubMed]
2. McNeely CA, Vassileva CM. Long-term outcomes of mitral valve repair versus replacement for degenerative disease: a systematic review. Curr Cardiol Rev. 2015;11(2):157-62. [CrossRef] [PubMed]
3. Christina MV, Gregory M, Christian M, Theresa B, Stephen M, Steven S, Stephen H. Long term survival of patients undergoing mitral valve repair and replacement a longitudinal analysis of Medicare fee-for-service beneficiaries. Circulation. 2013;127(18):1870–6. [CrossRef] [PubMed]

Cite as: Young R, Ravajy A. Medical image of the month: Massive right atrial dilation after mitral valve replacement. Southwest J Pulm Crit Care. 2018;18(1):8-9. doi: https://doi.org/10.13175/swjpcc111-18 PDF 

Medical Image of the Week: Cannon V Waves

Figure 1. Video showing jugular venous distention to earlobes with cannon V waves.

A 66-year-old man experienced recurrent ascites of unknown etiology over six months. He had previously undergone a renal transplant secondary to complications of diabetes and hypertension and had known severe coronary artery disease. His most recent paracentesis revealed an albumin 1.6 g/dL (serum albumin 2.1) and a total protein of 3.8 g/dL. His adenosine deaminase was 11.6 U/L (normal <7.6 U/L), but repeated bacterial and mycobacterial ascites cultures were negative, as were a carcinoembryonic antigen assay and ascites cytology. Computerized tomography of the abdomen showed findings consistent with cirrhosis, but an extensive workup for common causes of cirrhosis was negative.

Physical exam showed jugular venous distention with prominent V waves and a holosystolic murmur at the left lower sternal border (Figure 1). Echocardiography showed a dilated right ventricle, moderate pulmonary and tricuspid regurgitation and an estimated right ventricular systolic pressure of 87 mm Hg. Cardiac catherization confirmed the presence of an elevated right ventricular pressure of 72/10 (22) mm Hg, an elevated pulmonary artery pressure of 75/27 (45) mm Hg and a left ventricular ejection fraction of 20-25%. The right atrial pressure was 20 and the pulmonary artery occlusion pressure was 22 mmHg.  A diagnosis of pulmonary hypertension secondary to left ventricular heart disease (type 2 pulmonary hypertension) with congestive hepatopathy and cardiac ascites was made.

The patient’s physical examination provided an important clue to the etiology of the ascites – cardiac ascites is thought to be due to chronic venous congestion of the liver due to transmission of high central venous pressures. Tricuspid regurgitation can be associated with severe hepatic congestion because of retrograde transmission of right ventricular pressure directly into the hepatic veins. In some patients (although not in this patient), careful examination will reveal that the liver in such patients is palpably pulsatile.

Cardiac ascites is typically characterized by a serum albumin gradient (SAAG) >1.1 g/dL (indicative of portal hypertension) and ascites protein level of >2.5 g/dL (1). We cannot fully explain why this patient’s SAAG was low. A complete workup for infectious and oncological etiologies of low SAAG ascites was negative. It has been noted that in patients with known cirrhosis (as in this patient), the finding of a low SAAG has a low specificity for infectious and oncological etiologies of ascites (2). Serositis which can sometimes manifest as ascites can also be a complication of tacrolimus which the patient was receiving s/p renal transplant. It’s possible that tacrolimus might have changed the nature of the ascites fluid in this patient but this is conjectural. 

Robert A. Raschke, MD

College of Medicine-Phoenix

Phoenix, AZ USA

References

1. Sam AH, James THT. Rapid Medicine. Wiley-Blackwell; 2009: ISBN 1-4051-8323-3.
2. Khandwalla HE, Fasakin Y, El-Serag HB. The utility of evaluating low serum albumin gradient ascites in patients with cirrhosis. Am J Gastroenterol. 2009 Jun;104(6):1401-5. [CrossRef] [PubMed] 

Cite as: Raschke RA. Medical image of the week: cannon V waves. Southwest J Pulm Crit Care. 2017;15(2):90-1. doi: https://doi.org/10.13175/swjpcc095-17 PDF

Medical Image of the Week: Pulsus Alternans

Figure 1. Telemetry display including arterial pressure waveform, which demonstrates alternating beats of large (large arrows) and small (small arrows) pulse pressure. Concurrent pulse oximetry could not be performed at the time of the image due to poor peripheral perfusion.

A 52 year old man with a known past medical history of morbid obesity (BMI, 54.6 kg/m²), heart failure with preserved ejection fraction, hypertension, untreated obstructive sleep apnea, and obesity hypoventilation syndrome presented with increasing dyspnea over several months accompanied by orthopnea and weight gain that the patient had treated at home with a borrowed oxygen concentrator. On arrival to the Emergency Department, the patient was in moderate respiratory distress and hypoxic to SpO2 70% on room air. Physical examination was pertinent for pitting edema to the level of the chest. Assessment of jugular venous pressure and heart and lung auscultation were limited by body habitus, but chest radiography suggested pulmonary edema. The patient refused aggressive medical care beyond supplemental oxygen and diuretic therapy. Initial transthoracic echocardiography was limited due to poor acoustic windows but suggested a newly depressed left ventricular ejection fraction (LVEF) of <25%. The cause, though uncertain, may have been reported recent amphetamine use. The patient deteriorated, developing shock and respiratory failure; after agreeing to maximal measures, ventilatory and inotropic/vasopressor support was initiated.

Shortly after placement of the arterial catheter, the ICU team was called to the bedside for a change in the arterial pressure waveform (Figure 1), which then demonstrated alternating strong (arrow) and weak beats (arrow head) independent of the respiratory cycle. The waveform was recognized as pulsus alternans. Repeat bedside echocardiography suggested severe biventricular systolic impairment and LVEF of approximately 5-10%, later confirmed by formal transesophageal ehocardiography performed prior to a cardioversion for atrial flutter.

Pulsus alternans was first formally described in 1872 and associated with severe left ventricular systolic dysfunction (1). The pattern of pulsus alternans is detectable by palpation, arterial pressure waveform analysis, and Doppler echocardiography. Competing theories in the early 20^th century attempted to explain this finding. Wenkebach and Straub, using the Starling relationship, suggested that the alternating force of the pulse is due to alternating filling volumes: greater diastolic volumes accommodated by increased fiber length caused forceful contraction/greater stroke volume with subsequently reduced end systolic and therefore end diastolic volumes for the next (weaker) beat; the consequently reduced force left again greater end systolic and end diastolic volumes for the next (more powerful) beat thereafter. Gaskell, Hering, and Wiggers alternatively proposed the phenomenon was rooted in myocardial contractility fluctuations independent of volumes. Laboratory and animal data supported both theories, but seminal clinical work in the 1960s using concurrent ventriculography and ventricular pressure measurements demonstrated that both mechanisms, in fact, occur in different human subjects (2). The second, Starling-independent mechanism is now thought to be due at least in part to delayed intracellular calcium cycling leading to rhythmic fluctuations in excitation-contraction coupling (3).

Regardless of the underlying physiology, the significance of pulsus alternans as a harbinger of severe ventricular dysfunction and poor prognosis has been recognized and unquestioned since its description. This was unfortunately true in the case of our patient, who developed multiorgan failure despite resuscitative efforts and died three days after admission.

Luke M. Gabe, MD

University of Arizona College of Medicine

Department of Internal Medicine

Division of Pulmonary, Allergy, Critical Care and Sleep Medicine

1501 N. Campbell Ave.

Tucson, AZ USA

References

1. Traube L. Ein fall von pulsus bigeminus nebst bemerkungen tiber die lebershwellungen bei Klappenfehlern und über acute leberatrophic. Ber Klin Wschr. 1872;9:185.
2. Cohn KE, Sandler H, Hancock EW. Mechanisms of pulsus alternans. Circulation. 1967 Sep;36(3):372-80. [CrossRef] [PubMed]
3. Euler DE. Cardiac alternans: mechanisms and pathophysiological significance. Cardiovasc Res. 1999 Jun;42(3):583-90. [CrossRef] [PubMed]

Cite as: Gabe LM. Medical image of the week: pulsus alternans. Southwest J Pulm Crit Care. 2016;13(5):266-7. doi: https://doi.org/10.13175/swjpcc123-16 PDF 

Medical Image of the Week: Left Ventricular Non-compaction

Figure 1. Cardiac MRI showing severely enlarged and remodeled left ventricle (LV) and moderately enlarged right ventricle (RV) with severe global hypokinesis and akinesis of the interventricular septum. Significant trabeculation was noted in the apical, antero-lateral and anterior segments of the LV (red arrows), consistent with LV non-compaction.

A 38-year-old woman with history of type 2diabetes mellitus and hypertension presented to emergency department with worsening exertional dyspnea and orthopnea for the past 2-3 months. She also reported a 14 pound weight gain within the 2 weeks prior to presentation. She denied any prior history of cardiac or pulmonary disease. Also, there was no family history of heart disease. She denies any recent sick contacts, smoking, alcohol drinking, or substance abuse.

Physical exam revealed jugular venous pressure of 10 cm H₂O and significant bilateral lower extremity pitting edema. Chest x-ray showed an enlarged cardiac silhouette. Brain naturetic peptide (BNP) was 2,917 pg/mL. A subsequent echocardiogram revealed a left ventricular (LV) ejection fraction of 23% with severe global LV hypokinesia with moderate mitral regurgitation. Thyroid panel as well as iron panel were within normal range. Other laboratories were unremarkable. For the new onset systolic heart failure, a coronary angiography was performed, which demonstrated normal coronary arteries. The patient was diagnosed with non-ischemic cardiomyopathy and underwent a cardiac MRI, which showed severely enlarged and remodeled LV and moderately enlarged right ventricle (RV) with severe global hypokinesis and akinesis of the intraventricular septum. Moreover, a significant trabeculation was noted in the apical, antero-lateral and anterior segments of the left ventricle (Figure 1), consistent with “LV non-compaction” without any evidence of LV thrombus. The patient was started on diuretics and safely discharged with significant symptoms improvement.  

LV non-compaction is a cardiomyopathy characterized by altered myocardial wall with prominent left ventricular trabeculae and deep intertrabecular recesses (1). Some authors believe that non-compaction of the ventricular myocardium results from abnormal persistence of the trabecular layer  while others believe that altered regulation in cell proliferation, differentiation, and maturation during ventricular wall formation, resulting in hyper-trabeculation (2). Its prevalence in the general population is unknown but among patients undergoing echocardiography is estimated at 0.014 to 1.3 percent. In patients with heart failure, its prevalence has been reported as 3 to 4 percent (3). Patients with LV non-compaction may present with heart failure, arrhythmias, sudden cardiac arrest, syncope, and thromboembolic events. The diagnosis is usually established by transthoracic echocardiography. When echocardiography is indeterminate, cardiac MRI, computed tomography, or left ventriculography could be an alternative diagnostic modality. Data on treatment of LV non-compaction are limited, and there is no standard therapy established for this condition. Medical management depends on the clinical manifestations, LV ejection fraction, presence of arrhythmias, and risk of thromboembolism.

Rostam Khoubyari MD^1,2 and Seongseok Yun MD PhD³

¹Department of Cardiology, ²Sarver Heart Center; and the ³Department of Medicine, University of Arizona

Tucson, AZ USA

References

1. Maron BJ, Towbin JA, Thiene G, et al. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation. 2006 Apr 11;113(14):1807-16. [CrossRef] [PubMed]
2. Henderson DJ, Anderson RH. The development and structure of the ventricles in the human heart. Pediatr Cardiol. 2009 Jul;30(5):588-96. [CrossRef] [PubMed]
3. Kovacevic-Preradovic T, Jenni R, Oechslin EN, Noll G, Seifert B, Attenhofer Jost CH. Isolated left ventricular noncompaction as a cause for heart failure and heart transplantation: a single center experience. Cardiology. 2009;112(2):158-64. [CrossRef] [PubMed]

Cite as: Khoubyari R, Yun S. Medical Image of the week: left ventricular non-compaction. Southwest J Pulm Crit Care. 2016;12(6):229-30. doi: http://dx.doi.org/10.13175/swjpcc036-16 PDF

Medical Image of the week: ECHO findings of Apical Ballooning Syndrome

  Figure 1. Panel A: Four chamber view of the heart at the end of diastole with a dilated left ventricle. Panel B: Same four chamber view of the heart at the end of systole with a dilation and akinesis of the apical portion (arrow) of the heart consistent with apical ballooning/stress cardiomyopathy

A 79 year old man with a history of lung cancer. bladder cancer, chronic obstructive pulmonary disease, and coronary artery disease with two previous myocardial infarctions, presented to the emergency department with respiratory failure secondary to pulmonary edema. Further evaluation was significant for non-ST segment elevation myocardial infarction. Cardiac catheterization was remarkable for a focal, eccentric 95% stenosis of the proximal to mid left anterior descending artery that failed stenting due to extensive calcifications. An echocardiogram (ECHO, Figure 1) revealed an ejection fraction of 47% with akinesis of the mid to distal anterior, lateral, inferior, septal and apical segments consistent with takotsubo cardiomyopathy.

Takotsubo cardiomyopathy, aka apical ballooning syndrome or stress induced cardiomyopathy, is a subtype of heart failure typically defined by proposed criteria from the Mayo Clinic that includes: 1. transient hypokinesis, akinesis, or dyskinesis in the left ventricular mid segments with or without apical involvement frequently occurring, but not always, in context to a stressful trigger; 2. the absence of angiographic evidence of obstructive coronary disease or plaque rupture; 3. new ECG abnormalities (ST-segment elevation and/or T-wave inversion) or modest elevation in cardiac troponin; and 4. the absence of pheochromocytoma and myocarditis (1). It predominantly occurs in post-menopausal women in relation to unexpected emotional or physical stress. Characteristic ECHO findings demonstrate a symmetrical regional wall motion abnormalities extending equally into the apical inferior and lateral wall with overall global hypokinesia (Figure 1) (2).

Sachin Kalarn MSIV; Sophie Galson MD; Kristina Skinner DO; Ryan Nahapetian MD, MPH

University of Arizona

Tucson, AZ

References

1. Akashi YJ, Goldstein DS, Barbaro G, Ueyama T. Takotsubo cardiomyopathy: a new form of acute, reversible heart failure. Circulation. 2008;118(25):2754-62. [CrossRef] [PubMed]
2. Chockalingam A, Xie GY, Dellsperger KC. Echocardiography in stress cardiomyopathy and acute LVOT obstruction. Int J Cardiovasc Imaging. 2010;26(5):527-35. [CrossRef] [PubMed]

Reference as: Kalarn S, Galson S, Skinner K, Nahapetian R. Medical image of the week: ECHO findings of aprical ballooning syndrome. Southwest J Pulm Crit Care. 2015;10(4):150-1. doi: http://dx.doi.org/10.13175/swjpcc024-15 PDF