Case Presentation (00662100)

 51 yr-old female with multiple comorbidities (HTN, IBS, GERD, and recurrent UTIs)

 2 months Hx of dyspnea and chest discomfort

 Associated Symptoms: orthopnea, edema in LE, productive cough and abdominal pain.

 Underwent extensive work-up

Rule Out (On admission)
 Pneumonia

 Cardiac Heart Failure

 UTI
Work-up
 Chest X-ray: clear lungs, no pleural effusion. Normal cardiac silhouette.

 Bacterial Culture: Negative

 EKG: Normal

 Echocardiography: 18x22 mm mobile mass attached to the interatrial septum.

Results
Cardiac Tumors
BY JOHN Y N I COL A S ( M E D - I II ST U DENT)
CAR DI OVASCUL AR S URG ERY CLE R KS HIP
A M E R ICA N UN I V ERSITY OF BE I R U T, JA N UA RY 2 0 1 8
Outline
1. Introduction

2. Classification

3. Clinical Presentation

4. Diagnosis

5. Treatment
Introduction
 Cardiac Tumors Known since Middle Ages

 First successful resection in 1954 by Swedish surgeon Clarence Crafoord

 Diagnostic puzzle and therapeutic challenge

Classification

Benign

Primary

Malignant
Cardiac
Tumors

Secondary Metastatic
Classification: Primary Benign Tumors
Myxoma
 50 – 70% of all primary cardiac tumors

 Peak Age in 50s, women > men

 75% in L. Atrium, 18% in R. Atrium, and rarely in ventricles

 Mostly occurs in fossa ovalis; also in subendocardial tissue

or cardiac valves.

 Soft, gelatinous consistency. Soft surface covered with

thromboses.
Classification: Primary Benign Tumors
Myxoma
 90% are sporadic, with high recurrence rate.

 10% in Carney Syndrome (Autosomal Dominant):

o Cardiac and cutaneous myxomas

o Endocrine Hyperfunction, prolactinoma (case)

o Cutaneous Hyperpigmentation
Classification: Primary Benign Tumors
Endocardial Fibroelastosis
 Most Common Tumor affecting valves

 Average age  60 years-old

 Small, white, gelatinous mass

Lipoma
 Affects interatrial septum
Classification: Primary Benign Tumors
Rhabdomyoma
 Most common primary cardiac tumor in children

 Focal hamartomatous accumulation of striated cardiomyocytes

 Occurs in myocardium of the left ventricle or in the interventricular septum

 Associated with tuberous sclerosis, and congenital heart defects

 Regress spontaneously in 50% of patients.

Classification: Primary Malignant Tumors
 Sarcomas constitute 75% of all malignant cardiac tumors

 Mostly found in right atrium, but can occur in any chamber.

 Without treatment, life expectancy is only few months

Classification: Primary Malignant Tumors
Angiosarcoma
 Most Common, 30% of all malignant tumors
 Mostly in middle-aged men

Rhabdomyosarcoma
 Second most common
 Male = Female
 Found in any chamber (single or multiple) and may infiltrate cardiac valves
 Can spread and infiltrate pericardium, pleura and mediastinum
Classification: Primary Malignant Tumors
Fibrosarcoma
 Mainly in adults and may be multiple
 Arise in right atrium and display intracavitary growth, pericardium may be infiltrated.

Leiomyosarcoma
 All age groups, Male = Female
 Soft mass extending beyond the borders of the heart

Li-Fraumeni Syndrome
 Autosomal Dominant (mutation in TP53 gene on chromosome 17)
 In young patients, under age of 45.
Classification: Secondary Tumors
 Occur as result of peripheral tumors (mostly lung, breast, stomach, liver, colon, kidney)

 Spread by hematogenous or lymphogenous ways

 More frequent than primary tumors

 Unclear presentation, masked by the peripheral tumor

 Critical surgical management

Clinical Presentation
 General Symptoms
o Fever, fatigue, weight loss, nights weats, etc.

o Hemorrhagic pericardial effusion --> malignant tumors.

 Obstruction
o If close to valves --> mimics stenosis of mitral or tricuspid valves

o If infiltrating heart walls --> symptoms of hypertrophic or restrictive cardiomyopathy

o If expanding towards SVC --> SVC syndrome (facial swelling, head fullness, orthopnea, etc.)

o Mostly heart failure presentation

Clinical Presentation
 Arrhythmias
o If tumor infiltrating neural pathways or myocardium --> irregular heart beat, AV-block

o Mostly in fibromas

o Sudden Cardiac Death is sometimes the first presentation

 Embolisms
o Cardiac tumors are often first diagnosed after the patient has suffered a stroke

o Embolism of peripheral vasculature

o Pulmonary Artery Embolism

Diagnosis
 History
 Investigation Should begin with exclusion of a thrombus or a vegetation
 Echocardiography --> First diagnostic procedure
 If echo is non-confirmatory --> Try CT, MRI, or FDG-PET/CT (Benign Vs. Malignant)

Myxoma in Right Atrium Sarcoma in Right Nodular Recurrent

Atrium and Ventricle Sarcoma
Diagnosis
 In the absence of tumors elsewhere, a primary malignant cardiac tumor must be

assumed

 If Lymphoma suspected --> confirm by a tissue sample biopsy, followed by

chemotherapy and/or radiotherapy

 Coronary angiography helpful in determinig the extent of highly vascular tumors

Treatment
 Involves multidisciplinary team (oncologists, radiotherapists, and surgeons)

 No high level of evidence on the best treatment modality

 Surgical excision of the tumor is usually the treatment of choice

 Chemotherapy --> widespread or unresectable malignant disease

 Chemotherapy + Radiotherapy --> Primary Cardiac Lymphoma

Treatment: Simple Tumor Resection
 Mostly for Benign Tumors

 Care taken in connecting the heart–lung machine to avoid dislodging any tumor material

 Open both atria from the right superior pulmonary vein without injuring the tumor or its base

 Tumor and its root can then be removed from the septum

 Inspect all the chambers of the heart to exclude the presence of further tumors
Treatment: Complex Tumor Resection
 If tumor on right side is too advanced --> Resect right side

 Pulmonary blood flow assured by Fontan Circulation

 Chronic right heart failure may result

 Treatment: Ex-situ Resection
 Heart can be removed from the thorax to facilitate complete
resection

 Done when tumor involves posterior wall of left atrium or dorsal

great vessels.

 Better exposure --> Complete resection and better long-term

outcome

 After resection, cardiac anatomy is restored with artificial

materials and heart is replanted
 Treatment: Myxoma
 Classical approach --> surgical exploration through the left atrium

 Incision --> at the interatrial groove

 Inspection of the attachment to arterial wall or atrial septum

 Right atrium is then opened, if needed

Treatment: Fibroma
 Tumor resected from trans-tricuspidal and
transventricular approach

 Locate tumor on ventricular wall + enucleation

from wall

 Suture the endocardial layer to epicardium to

avoid intraparietal hematoma formation

 Gore-Tex patch necessary to close defect left

in anterior wall of right ventricle
Treatment: Endoscopic Robotic Resection
 Small incisions under the right armpit lateral to

the breast

 Biggest chest incision is the working port which is

15 mm in size

 Same level of effectiveness but is far less

invasive
Treatment: Endoscopic Robotic Resection

 Procedure does require cardiopulmonary bypass

 Peripheral catheter-based system

 Tumor is resected and removed using an “endobag”

Treatment: Endoscopic Robotic Resection

https://www.youtube.com/watch?time_continue=73&v=PK8o-udtqas
Treatment: Endoscopic Robotic Resection
Benefits of Endoscopic Robotic Resection:
o Less painful

o Faster Recovery

o Shorter hospital stay

o Sternum and chest bones intact --> less complications, less infections

o Minimal blood loss, less need for blood transfusions

Treatment: Endoscopic Robotic Resection
Risks of Endoscopic Robotic Resection:
o Need to convert to open techniques
o Aortic Dissection (tearing of the layers of aorta)
o Strokes
o Injury to the liver

Risks associated with bypass in robotic surgery:

o Injury to leg artery or vein
o Deep Vein Thrombosis
o Lymphocele (collection of fluid in groin area)
Thank you.