Interesting Cases

January 2014

History

The patient is a 69 year old female with significant past medical history of hypertension, hyperlipidemia, type2 diabetes mellitus, obesity, and atrial fibrillation. She presents to clinic with an episode of chest discomfort (resolved spontaneously) and new onset murmur discovered by PCP. She denies dyspnea, syncope, or signs and symptoms of heart failure. She denies fevers, night sweats, or any previous cardiac surgery.

Echo (TTE)

  • Echogenic mass (0.9 cm x 1.1 cm) noted on aortic side of the aortic valve
  • Precise mass attachment point uncertain – non-­coronary cups of the aortic valve vs. proximal aortic wall

Differential

Myxoma Papillary Fibroelastoma Lipoma Thrombus Endocarditis Lambl’s
Incidence Up to 50% intra-cardiac tumors Up to 8% intra-cardiac tumors 2nd most common intra-cardiac Common with: Prosthetic Valves (mech >>biop)1 LVAD, or ECMO More common prosthetic valve 40%
Location IAS attachment Valvular endocardium Anywhere Valve location is RARE2 Uncommon on valve unless above conditions Left-sided lesions 90% of cases Aortic and/or ventricular surface of aortic valve
Echo Heterogeneous Broad based attachment Homogenous with stalk3 Homogenous, Encapsulated No clear point of attachment Echogenicity different than myocardium Move in phase with valves since History important in differentiating from endocarditis and fibroelastoma
Addtnl Frequent presentation: dyspnea, chest pain, fatigue.4 75% of valvular tumors are papillary fibroelastomas.5 Case report of man with protein C deficiency as presumptive cause of Aortic Thrombus.6 Clinical context important in diagnosis Thought to be associated with stroke

Video 1: AV LAX showing mobile mass attached to the left or non-­coronary cusp
Video 2: AV SAX showing mobile mass attached to the non-coronary cusp
Video 3: AV Short Axis – 3D view of the aortic valve from the ascending aorta showing mobile mass attached to the non-coronary cusp
Video 4: AV Short Axis – 3D Cropped showing attachment point of the mobile mass at the base of the non-­coronary cusp

Pathology

1.1 x 0.9 x 0.5 cm tan, gelatinous soft portion of tissue
Diagnosis Papillary Fibroelastoma

References

  1. Ohnaka M, Nishimura K, Kurokawa S. Flat fibrin thrombus deposition on tissue valve after aortic valve replacement. The Annals of thoracic surgery. Jun 2010;89(6):2032-­‐2034.
  2. Matsushita T, Huynh AT, Singh T, et al. Aortic valve lipoma. The Annals of thoracic surgery. Jun 2007;83(6):2220-­‐2222.
  3. Seto T, Takano T, Otsu Y, et al. Cardiac Papillary Fibroelastoma: Report of Three Cases. Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia. Oct 3 2013.
  4. Burnside N, MacGowan SW. Malignant primary cardiac tumours. Interactive cardiovascular and thoracic surgery. Dec 2012;15(6):1004-­‐1006.
  5. Edwards FH, Hale D, Cohen A, Thompson L, Pezzella AT, Virmani R. Primary cardiac valve tumors. The Annals of thoracic surgery. Nov 1991;52(5):1127-­‐1131.
  6. Yokoyama Y, Satoh H, Kurata A, et al. [Surgical removal of native aortic valve thrombosis associated with acute myocardial infarction and protein C deficiency; report of a case]. Kyobu geka. The Japanese journal of thoracic surgery. Mar 2009;62(3):238-­‐240.

January 2014

Persistent left superior vena cava (PLSVC):

  • Most common variation in the thoracic venous system
  • Present in 0.5% of the population. Can be associated with other congenital anomalies such as: bicuspid aortic valve, atrial septal defect, or coarctation of the aorta
  • Can be concomitant with absence of right superior vena cava (SVC) in which case the right internal jugular vein and the right subclavian vein empty through an innominate vein in the PLSVC.
  • In 10% of the cases PLSVC empties directly into the left atrium resulting in a small left-­to-right shunt. • In most cases PLSVC empties into the right atrium through the coronary sinus. In this situation, PLSVC is associated with a dilated coronary sinus, diameter greater than 1 cm (Video 1 shows a dilated coronary sinus in a modified midesophageal bicaval view). Injection of agitated saline into a venous line situated in the left superior extremity or left neck will result in opacification of the coronary sinus before the opacification of the right atrium (Video 2).
  • Normally, the left innominate vein travels laterally to the aortic arch in a parallel fashion. The PLSVC travels laterally to the aortic arch at an orthogonal angle (Video 3 shows an X plane view of the aortic arch; note the dissection flap in the aortic arch and the PLSVC in short axis at 0° and in long axis at 90° adjacent to the aortic arch).
  • The PLSVC course then travels between the left atrial appendage and the left upper pulmonary vein (Video 4 and Video 5 show an echo lucent space with agitated saline (Video 5) between the left atrial appendage and the left upper pulmonary vein where the ligament of Marshall—which is the fibrosed left SVC—should be)
  • The presence of PLSVC has several implications in (1) placement of a central line, (2) floating of a pulmonary catheter, (3) administration of retrograde cardioplegia , and (4) venous drainage during cardiac surgical procedures on the right heart.
  • Myxomas are the most common primary neoplasm of the heart, with an incidence of 0.5 per million population per year, and they account for 50% of all primary cardiac tumors diagnosed at the time of autopsy. Myxomas are most commonly diagnosed in the 3rd-6th decade and have a 65% female predominance. Up to 10% of myxomas are associated with familial syndromes such as Carney complex.
  • Patients may be asymptomatic, or may present with constitutional, embolic or obstructive symptoms. Approximately 75-85% of myxomas occur in the left atrium, 5-20% occur in the right atrium, and a small percentage are found in the ventricles or in multiple locations. On TEE, myxomas usually appear as sessile or pedunculated heterogeneous structures and are most commonly attached by a stalk to the interatrial septum in the region of the fossa ovalis. Mxyomas may prolapse through the atrioventricular valve and can cause obstruction or damage to the valve.
  • The pre-procedure TEE exam should focus on classifying the tumor and attachment site (using 3-D if necessary), evaluating the associated valve for functional stenosis or valvular damage and ensuring multiple myxomas are not present. The post-CPB exam should focus on ensuring a complete resection has taken place and verifying that no residual septal defect or valvular insufficiency is present.

Video 1 Midesophageal 4-chamber view with color flow Doppler shows large myxoma prolapsing through and obstructing the mitral valve leading to functional mitral valve stenosis.

Video 2 Midesophageal long axis view with color flow Doppler

Video 3 Midesophageal 2-chamber view showing attachment of the myxoma not at the level of the fossa ovalis but rather in the vicinity of the left atrial appendage, possible ligament of Marshall.

Video 4 3D- zoom view of the myxoma

Video 5 En-face 3D view of the mitral valve showing large myxoma occupying almost entirely the mitral valve area.

Reference

Reynen, K Cardiac myxomas N Engl J Med 1995; 333:1610-1617

Most common congenital anomaly at birth but only 10-­‐15% of defects in adults with congenital heart disease.

Type AKA Description % of all VSDs
Supracristal
  • Subarterial
  • Outlet
  • Subpulmonic
  • Doubly committed
  • Infundibular
  • Usually circular and located within infundibular portion of RVOT
  • Superior edge of VSD is conjoined annulus of aortic and pulmonary valves
  • Both outlet septum and septal component of subpulmonary infundibulum are absent
5
Infracristal
  • Perimembranous
    • Perimembranous inlet
      • Primarily adjacent
        to TV
      • LVOT→RV beneath
        septal cusp
    • Perimembranous outlet
      • Defect extends primarily toward aortic valve
  • Involves membranous septum
  • Below AV and lateral to septal TV leaflet
70
Muscular Located in central or apical trabecular portion of the septum 20
Atrioventricular
canal
  • inlet
Posterior to membranous IVS between TV/MV

  • usually caused by defect in formation of AV septum
  • can be associated with a cleft in MV or TV
5
VSD Peak pressure (mmHg) LA or LV dilatation Pulmonary artery pressures
Restrictive > 75 No Normal
Mod Restrictive 25-75
Nonrestrictive < 25 ↑↑ ↑↑

VSD-heart

  • 10% of perimembranous defects can undermine right aortic cusp causing herniation of cusp and AI
  • Gerbode defect – rarely a perimembranous VSD can lead to communication between the LV outflow tract and right atrium
  • Small membranous VSDs may close spontaneously during childhood by approximation of TV septal leflet across the defect
  • Crista supraventricularis is synonymous with infundibular septum (muscle separating outflow tracts of L and R ventricles)

Echocardiographic Assessment

Type AKA Echocardiographic View
Supracristal
  • Subarterial
  • Outlet
  • Subpulmonic
  • Doubly committed
  • Infundibular
Midesophageal RV inflow
outflow (figure 1-A)
Infracristal
  • Perimembranous
    • Perimembranous inlet
      • Primarily adjacent to TV
      • LVOT→RV beneath septal cusp
    • Perimembranous outlet
      • Defect extends primarily toward aortic valve
Midesophageal 5-chamber
Midesophageal long axis
Midesophageal RV inflow
outflow (figure 1-B)
Muscular Midesophageal 4-chamber
Transgastric short-axis (basal, mid-papillary or apical)
Atrioventricular canal
  • inlet
Midesophageal 4-chamber
Figure1: ME RV inflow-­‐outflow. A: supracristal (subarterial, outlet, subpulmonic, infundibular) B: infracristal (perimembranous, subaortic)

Figure1: ME RV inflow-­‐outflow. A: supracristal (subarterial, outlet, subpulmonic, infundibular) B: infracristal (perimembranous, subaortic)

Video 1: Midesophageal RV inflow­outflow with color flow Doppler shows a small residual perimembranous VSD after patch repair.

Video 2: Non-­standard midesophageal mitral commissural view with the probe turned to the right shows blood flow through an ischemic muscular VSD opening under the tricuspid valve.

Video 3: Same patient as video 2. Transgastric short axis midpapillary view with color flow Doppler shows blood flow through a complex ischemic muscular VSD at the level of the inferoseptal wall. The VSD seems to open underneath the tricuspid valve at the level of the posteroseptal commissure.

Video 4: Same patient as video 2 and 3. The patient underwent repair of the VSD with patch with approach through the right atrium and the tricuspid valve. The tricuspid valve was also replaced as the tricuspid valve had to be removed to facilitate access to VSD. Non-­standard midesophageal mitral commisural view with the probe turned to the right shows the result of the patch repair.

Echo Faculty

Solomon Aronson, MD
Brandi Bottiger, MD
Anne Cherry, MD
Brian Colin, MD
J. Mauricio Del Rio, MD
Nicole Guinn, MD
Jorn Karhausen, MD
Miklos Kertai, MD
Rebecca Klinger, MD
Yasmin Maisonave, MD
Michael Manning, MD
Joseph Mathew, MD
Alina Nicoara, MD
Mihai Podgoreanu, MD
Mark Stafford-Smith, MD
Madhav Swaminathan, MD
Eleanor Vega, MD
Ian Welsby, MB

Staff

Jaime Cooke, Staff Assistant
Audrey McGregor, Staff Assistant
Dheadra McAdoo, Echo Technician
Cheryl Stetson, CT Fellowship Coordinator

Links

ChrisInteresting Cases