Pericardial Surgery and Cardiac Tumors

Question 1

Blood Supply of the Pericardium

Answer 1

1. Pericardiophrenic arteries
2. Branches if the IMAs
3. Branches directly from the Aorta

Question 2

Innervation of the Pericardium

Answer 2

1. Vagal Fibers from the Esophageal Plexus
2. Phrenic Nerve Branches

Question 3

Pericardial Sinuses

Answer 3

1. Oblique Sinus - pulmonary venous confluence
2. Transverse Sinus - between the great arteries and dome of the LA and RPA

Question 4

Normal Pericardial Fluid Volume

Answer 4

10-20mL

Question 5

Ventricular Interdependence

Answer 5

Intrapericardial pressure reflects intrathoracic pressure. During inspiration intrathoracic pressure and PVR decreases increasing RV preload causing a leftward shift of the IVS resulting in decreased LV preload and resultant SV reflected by a decrease in BP.

Question 6

Pulsus Paradoxus

Answer 6

Exaggerated Ventricular Interdependence

(during inspiration PVR decreases increasing RV preload causing a leftward shift of the IVS resulting in decreased LV preload and resultant SV reflected by a decrease in BP)

Greater than 10mmHg drop in SBP during inspiration.

Question 7

Describe the changes to the JVP trace for

Tamponade

vs.

Constrictive Pericarditis

vs.

Restrictive Cardiomyopathy

Answer 7

Tamponade

Prominant ‘‘x”

Blunted “y”

Constrictive Pericarditis

Prominant “x”

Prominant “y”

Restrictive Cardiomyopathy

Blunted “x”

Prominant “y”

Question 8

Partial or Complete Agenesis of the Pericardium

Answer 8

Partial Absence of the Pericardium

• 70% Left
• 20% Right; Usually Complex and Lethal

Complete Agenesis of the Pericardium

• Rare
• Usually asymptomatic and of no consequence
• Can result in incarceration of the LAA or LV due to excessive mobility within the chest

Question 9

Pericardial Cysts

Answer 9

Most Common Peridarial Disorder

70% Occur at the Right Costalphrenic Angle

Usually Incidental Finding

Symptoms (related to complession or inflammation)

1. Pain
2. SOB
3. Cough
4. Arrhythmias

Indications for Resection

1. Large Size
2. Symptoms
3. Patient Concern
4. Question of Malignancy

Question 10

Beck’s Triad

Answer 10

Clinical Manifestations of Tamponade

1. HoTN
2. Jugular Venouse Distention
3. Muffled Heart Sounds

Question 11

Compenatory Responses to Increased Diastolic Filling Pressures

Answer 11

1. Parallel Increase in PVR and SVR
2. Tachycardia
3. Chronic Pericardial Stretch
4. Increased Preload (expand blood volume)

Question 12

Symptoms of Pericardial Constriction

Answer 12

1. Fatigue
2. Exercise Intolerance
3. Dyspnea/Orthopnea
4. Peripheral Edema
5. Ascites

Question 13

Pathophysiology of Pericardial Constriction

Answer 13

• Impairment of Late Diastolic Ventricular Filling
• Ventricular Interdependence causes an abrupt leftward shift of IVS (Septal Bounce) and Pulsus Paradoxus

Question 14

Kussmaul’s Sign

Answer 14

JV distension during inspiration

Question 15

Echocardiographic Findings of Pericardial Constriction

Answer 15

1. Septal Bounce
2. Pericardial Thickening
3. Plethoric IVC
4. Underfilled Chambers

Question 16

Hemodynamic Indicators of Pericardial Constriction on Cath

Answer 16

1. Square Root Sign

Sudden increase in RV filling pressure in late diastole followed by equilization of diastolic filling pressures

2. Steep “x” and “y” descent (Restrictive will have blunted “x”)
3. ***Ventricular Interdependence

discordant increase in RV and decrease in LV pressure curve during inspiration

Question 17

Constrictive Pericarditis vs. Restrictive Cardiomyopathy

Answer 17

Constrictive Pericarditis

• Dissociation of intrathoracic and intracardiac filling pressures measured by respiratory variation in the gradient between the LVP and PCWP during the rapid filling phase of diastole
• ***Ventricular Interdependence - discordant increase in RV and decrease in LV pressure curve during inspiration

Question 18

Causes of Pericarditis

Answer 18

1. Infectious (viral, bacterial, fungal)
2. Metabolic (uremia, drug induced)
3. Autoimmune (arthritis, thyroid)
4. Post-Radiation
5. Neoplastic
6. Traumatic
7. Post-Infarct (Dressler’s Syndrome; 10-15%)
8. Postcardiotomy Syndrome (5-30%)
9. Idiopathic

Question 19

ECG Findings of Pericarditis

Answer 19

1. Diffuse ST elevation (without reciporical changes or Q-waves)
2. PR depression

Question 20

Trials on the Treatment of Pericarditis

Answer 20

ICAP

• RCT; NSAIDs vs. Colchicine for First Episode Pericarditis
• Reduced Persistence and Reccurence

CORP and CORP-2

• NSAIDs vs. Colchicine
• Reduced Recurrence

Question 21

Most Common Bacteria Causing Pericarditis

Answer 21

1. Haemophilus Influenzae
2. Meningococci
3. Pneumococci
4. Staphylococci
5. Streptococci
6. Salmonella
7. TB (developing nations or HIV)

Question 22

Fungal Causes of Pericarditis

Answer 22

1. Nocardia
2. Aspergillus
3. Candida
4. Coccidioides
5. Histoplasma

Question 23

Drugs Associated with Pericarditis

Answer 23

1. Hydralizine
2. Procainamide
3. Isonaizid
4. Methysergide
5. Cromolyn
6. Penicillin
7. Emetine
8. Minoxidil

Question 24

Most Common Secondary Tumors of the Pericardium

Answer 24

Males

1. Carcinoma of the Lung (30%)
2. Carcinoma of the Esophagus (30%)
3. Lymphoma (10%)

Females

1. Carcinoma of the Lung (35%)
2. Lymphoma (20%)
3. Carcinoma of the Breast (10%)

Question 25

**Risk Factors** for **Postop** Pericardial **Effusion**

Answer 25

1. Increased **BSA** 2. **PE** 3. **Immunosuppression** 4. Surgery (**Transplant** or **Aortic** Aneurysm) 5. Long **CPB Time** 6. **Urgency** 7. **Renal** Failure

Question 26

Findings of **Postoperative Tamponade**

Answer 26

1. Early Postop **Bleeding** with Decreased or No **CT Output** 2. **Tachycardia** 3. Narrow **Pulse Pressure** 4. **Pulsus Paradoxus** 5. Increased **CVP** 6. **Oliguria** 7. **Acidosis** 8. Elevated **Lactate** 9. Escalating **Inotropes**/**Vasopressors** 10. Decreased **Cardiac Index** 11. Decreased **MVO₂** 12. **Electrical alternans** 13. Pericardial **effusion**

Question 27

**Management** of **Postoperative** **Tamponade**

Answer 27

Correct... 1. **Coagulopathy** 2. **Hypothermia** 3. **Acidosis** 4. **Hypovolemia** Urgent **Surgical** Mediatrinal Exploration

Question 28

**Outcomes** following Emergent Mediastinal Exploration in the **ICU** for **Tamponade**

Answer 28

Perioperative **Mortality** - **85%** Sternal Wound **Infection** - **5****%**

Question 29

**Echo**cardiographic Findings of **Tamponade**

Answer 29

1. Early **Diastolic** **RV** Collapse 2. Early **Systolic** **RA** Collapse 3. **IVC** Dilitation (\>50%) 4. Increased V**entricular Interdependence** with respiration 5. **Decreased** mitral inflow with **inspiration**

Question 30

**Approaches** to the **Pericardial Window**

Answer 30

1. Thorascoscopy 2. Anterior Thoracotomy 3. Subxiphoid

Question 31

**Waffle** Procedure

Answer 31

* For Constrictive Pericarditis of the **Viseral Pericardium**/Epicardium * **Longitudinal** and **Transverse Incisions** in the Epicardium 1cm Apart

Question 32

**Principle** and **Margins** of Dissection for Surgical **Pericardiectomy**

Answer 32

**Principle** * **Complete** Pericardial Resection **Margins** 1. **Anterior** from **Phrenic-to-Phrenic** Nerve 2. **Posterior** from the Reflection Around the **Vena Cava** to the **Pulmonary Veins**

Question 33

**Outcomes** Following Surigcal **Pericardiectomy**

Answer 33

**Operative** Mortality **5-10%** **1-year** Survival **90%** **10-year** Survival **85%** **\***Except Post-**Radiation** Constrictive Pericarditis - **60**/**0%**

Question 34

Frequency of **Benign** vs. **Malignant** **Primary** Cardiac Tumors

Answer 34

**75%** Benign **25%** Malignant (of which **75%** are **sarcomas**)

Question 35

Pathological Origins of **Cardiac Tumors**

Answer 35

1. Pseudotumors (**thrombus**) 2. Heterotopias/**Ectopic** Tissue Tumors (AV Node, Teratomas, Thyroid Tumors) 3. **Mesenchymal** (Endocardial Hamartomas, Papillary Fibroelastoma) 4. **Myocardial Hamartomas** (Rhabdomyoma, Purkinje Cell) 5. **Fat** (Lipomatous hypertrophy of the IAS, Lipoma, Liposarcoma) 6. **Fibrous** or **Myofibroblastic** Tissue (Fibroma, Fibrosarcoma, Leiomyosarcoma) 7. **Vascular** Tumors (Hemangioma, Angiosarcoma) 8. Mix/Unknown Histogenesis (**Myxoma**) 9. **Neural** Tumors (Granular Cell, Schwannoma/Neurofibroma) 10. **Paraganglioma** 11. **Lymphoma** 12. **Mesothelioma**

Question 36

Frequency of **Benign** Cardiac Tumors in **Adults**

Answer 36

1. **Myxoma** (50%) 2. **Lipoma** (20%) 3. **Papillary Fibroelastoma** (20%) 4. Hemangioma (5%) 5. AV Node Mesothelioma (5%) 6. Fibroma 7. Teratoma 8. Granualr Cell Tumor 9. Neurofibroma 10. Lymphangioma 11. **Rhabdomyoma** (\<1%)

Question 37

Frequency of **Benign** Cardiac Tumors in **Children**

Answer 37

1. **Rhabdomyoma** (50%) 2. **Teratoma** (20%) 3. **Fibroma** (15%) 4. **Myxoma** (15%) 5. Hemangioma 6. AV Node Mesothelioma 7. Neurofibroma

Question 38

**Myxomas**

Answer 38

* **50%** of **Adult** Cardiac Tumors * **15%** of **Childhood** Cardiac Tumors (Rare in infants) * **F** \> **M** * Peak Incidence **3-6th Decade** * **Solitary** (95%) * **75%** in **Left Atrium** (10-20% Right Atrium) * Right atrial myxomas have a higher association with multiple lesions * Low Recurrence Rate (1-5%) * Majority **Sporatic** (5% Familial)

Question 39

**Familial** Myxomas

Answer 39

* **5%** of Myxomas * **Autosomal Dominant** * Abnormal Chromosomal Genotype * Present at **Younger** Age * **F = M** * 20% **Multiple Tumors** in the Atria or Ventricles * **Higher Recurrence** Rate (20-60%) * **20% Complex Myxomas** with Assoc. Conditions 1. Adrenocortical Nodule Hyperplasia 2. Sertoli Cell Tumors 3. Pituitary Tumors 4. Multiple Myxoid Breast Fibroadenomas 5. Cutaneous Myomas 6. Facial or Labial Pigmented Spots

Question 40

**Carney's Syndrome**

Answer 40

Familial Syndrome **Autosomal Dominant** (chromosome 17) **X-Linked** Inheritance **M \>** F **Primary Pigmented Nodular Adrenocortical Disease** 1. **Hypercortisolism** 2. **Cutaneous Pigmentous Lentigines** and **Blue Nevi** 3. Cardiac **Myxomas** 4. Other endocrine tumors 1. **Thyroid** 2. **Testes** 3. **Ovaries** 4. **Pituitary Adenoma**

Question 41

Common **Presentations** of **Myxomas**

Answer 41

**CHF** (Obstruction) 60% **Embolization** 30% **Constitutional** Sx 20% Death occurs from obstruction or emoblization in 8% of paitents awaiting surgery

Question 42

**Surgical** Approach to **Myxoma** Excision

Answer 42

Intraoperative **TEE** to check for other assoc. tumors **Bicaval** cannulation **Cross clamp** the aortia prior to manipulating the LA **Transmural** excision of the stalk/attachment **Wall suction** wil tumor exposed to prevent systemic embolization **Mortality** risk **5%** (10% if ventricular myxoma)

Question 43

**Lipomas**

Answer 43

Encapsulated **Pericardium** **Subepicardium** assoc. with pericardial effusions **Subendocardium** **Intra-atrial septum** assoc. with compressive/obstructive Sx or arrhythmias M=F Most commonly **RA** or **LV**

Question 44

**Lipomatous Hypertrophy** of the Intra-Atrial Septum

Answer 44

**Non**-encapsulated 1. **Elderly** 2. **Obese** 3. **Females** Benign, may be assoc. with arrhythmias **MRI** helpful for tissue characterization for diagnosis

Question 45

Papillary **Fibroelastomas**

Answer 45

Heart **valves** or adjacent endocardium **Asymptomatic** until they **embolize** or obstruct (usually coronary ostia) **AV=Semilunar** valves

Question 46

**Rhabdomyomas**

Answer 46

Most frequent tumor in **Children** **Sporadic** Assoc. with **Tuberous Sclerosis** 1. Epilsepsy 2. Mental retardation 3. Sebaceous Adenomas **Ventricles** \> Atrium 90% **Multi-site** involvement

Question 47

**Presentation** of **Rhabdomyomas**

Answer 47

Valvular or subvavlular **stenosis** **Ventricular Tachycardia** Sudden **Death**

Question 48

**Surgery** for **Rhabdomyomas**

Answer 48

Surgical **resection with enucleation** before **1-year** of **NO** tuberous sclerosis **Tuberous sclerosis** assoc. with multiple lesions and has a very **poor prognosis**

Question 49

**Fibromas**

Answer 49

2nd most common **benign** tumor **80**% in **children** **M=F** Occur in the **Ventricule** or Ventricular **Septum** **Non**-encapsulated elongated fibroblasts

Question 50

**Presentation** of **Fibromas**

Answer 50

Chamber **obstruction** **Contractile dysfunction** **Arrhythmias** Sudden **Death** (**25**%)

Question 51

**Surgical** Resection of **Fibromas**

Answer 51

Resection/**enucleation** recommended if **vital structures NOT involved** **Complete** resection is **curative** If vital structures are involved then **partial resection** can be performed but is **palliative** **Transplantation** is an option

Question 52

**Mesothelioma** of the AV Node

Answer 52

AKA... ​ 1. **Polycystic** tumor 2. **Purkinje** tumor 3. **Conduction** tumor Presents with... 1. **AV block** 2. **Ventricular fibrillation** 3. Sudden **death**

Question 53

**Pheochromocytomas**

Answer 53

**Chromaffin cells** of the sympathetic nervous system Produce catecholamines (**norepinephrine**) **Young Adults** **M=F** 60% in the **roof** of the **LA**

Question 54

**Paragangliomas**

Answer 54

Endocrine tumor Secretes **catecholamines** Most commonly in the **posterior mediastinum** High **vascularity** contributes to risk assoc. with surgical resection

Question 55

**Teratoma**

Answer 55

Most common in **infants** and young **children** 80% **Benign**

Question 56

**Castleman Tumor**

Answer 56

**Lymphoproliferative** Disorder Histological types 1. **90**% **Hyaline** Vascular (**benign**) 2. **10**% **Mixed**-cell type (**malignant**)

Question 57

Primary malignant tumors (**sarcomas**)

Answer 57

Histological Types 1. **Angio**sarcomas 2. **Rhabdomyo**sarcomas 3. Malignant **mesotheliomas** 4. **Fibro**sarcomas Anatomical Types 1. Right sided (most angiosarcomas; infiltrative; metastisize early; neoadjuvant chemo) 2. Left sided (more solid; present with CHF; mistaken as myxomas; rapidly recure) 3. PA (present with R-sided HF/PE Sx; better prognosis) **Survival** only **10**% at **1-year**

Question 58

**Angiosarcomas**

Answer 58

**M \>** F **80**% **RA** **Invade** adjacent structures Present with **Obstruction** and **right-sided HF** Histology - **vascular channels** lined with **typical anaplastic epithelail cells** **Metastasize** to the lung/liver/brain 90% **dead within 9-12 months** of Dx

Question 59

**Malignant Fibrous Histiocytomas**

Answer 59

Histology - **fibroblasts** of **histioblasts** - mixture of **spindle** cells in a **storiform** pattern and **polygonal** cells Often mistaken for myxomas Patients die of **local recurrence** more commonly than metastatic disease

Question 60

**Rhabdomyosarcomas**

Answer 60

**M = F** Commonly **multicentric**, arising in both **ventricles** **Invasive** Histology - **pleomorphic nuclei**, spidery streaming **eosinophilic cytoplasm**, **muscle-like** pattern **Survival \< 12-months**

Question 61

**Secondary/Metastatic** Cardiac Tumors arise from what types of cancer?

Answer 61

1. **Leukemia** 2. **Melanoma** 3. **Lung** cancer 4. **Sarcoma** 5. **Breast** cancer 6. **Esophageal** cancer 7. **Ovarian** cancer 8. **Kidney** cancer 9. **Gastric** cancer 10. **Prostate** cancer 11. **Colon** cancer 12. **Lymphoma**

Question 62

Most common **cardiac** sites of **metastasis**

Answer 62

1. **Pericardium** 2. **Epicardium** 3. **Myocardium** 4. **Endocardium**

Question 63

Frequency of **Renal Cell Carcinoma** invasion of the **IVC** and **RA**

Answer 63

Invades the **IVC** in **10**% of cases Reaches the **RA** in **40**% of these cases If renal primary fully **resectable** **survival** is **75**% at **5-years**