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Table 6 Cancer therapy-related MACCE events in the cohort, adjudicated as cardiotoxicity events

From: Outcomes of malignancy in adults with congenital heart disease: a single center experience

Patient ID

ACHD diagnoses

MACCE prior to cancer therapy

Primary cancer

Cancer Therapy

Therapy-related MACCE

Time of therapy-related MACCE episode from initiation of therapy

Management of suspected cardiotoxicity

Outcome of cardiotoxic episode

Cancer-related outcome

3

• ASD

• VSD

• Subaortic membrane

None

Melanoma

Ipilimumab/ nivolumab

New non-ischemic cardiomyopathy with heart failure. LVEF decreased from 53 to 36% after first cycle of immunotherapy

31 days

• Ipilimumab discontinued, switched to trametinib.

• Furosemide, carvedilol, spironolactone and lisinopril started

• Improvement in LV EF and volume status.

• Able to tolerate additional cycles of therapy

• New brain metastases discovered

• Suffered large intracranial hemorrhage due to gamma-knife treatment

• Died shortly after comfort care initiated

11

• Bicuspid aortic valve

• Mitral valve prolapse

• SVT, paroxysmal AF

• Known LV cardiomyopathy (LVEF 35%) due to ventricular pacing/ mid-LAD 70% plaque

Mantle cell lymphoma

Bendamustine/ rituximab (BR)

Decompensated heart failure, with 1.2 L administration of fluids with first cycle

Same day as initiation

Prophylactic furosemide before each subsequent cycle

• Fluid status well-managed, no further decompensation or change in LV function.

• Completed 4 cycles of BR.

Remission followed by relapse

11

• Bicuspid aortic valve

• Mitral valve prolapse

Same as above

Mantle cell lymphoma

Ibrutinib

Decompensated heart failure. No changes to LV EF or valve function

30 days

• Discontinuation of ibrutinib,

• Initiation of bendamustine/ rituximab (BR) with scheduled hospitalization for management of fluid status with each dose

• Fluid status improved.

• Subsequent doses of BR given with intravenous furosemide during scheduled admissions

• Tolerated BR therapy well

• Progression to blast crisis

• Died shortly after comfort care initiated

21

Bicuspid aortic valve

None

Hodgkin’s lymphoma

Mediastinal radiation

• Radiation pericarditis

• Radiation induced severe valvulitis of the aortic, tricuspid and mitral valves

Unknown

• Pericardial window

• surgical tricuspid valve replacement.

• Worsening heart failure due to severe mitral and aortic regurgitation.

• Deemed unintervenable due to poor functional status from metastatic lung cancer

• Hodgkin’s lymphoma with remission

• Metastatic small-cell lung cancer diagnosed approximately 35 years later, progressed despite immunotherapy.

• Died shortly after being placed on palliative care for advanced cancer and heart failure

41

• Double outlet right ventricle (unintervened)

• Pulmonic stenosis

• VSD

Paroxysmal AF

Urothelial carcinoma

Gemcitabine/ cisplatin

• Acute chest pain with elevated troponin-I level to 0.86 ng/mL in setting of gastrocnemius vein thrombosis.

• No pulmonary embolism found

28 days

Conservative management advised since symptoms resolved.

• None, no recurrence in symptoms and hence no additional ischemia evaluation pursued

• Received another 2 cycles of gemcitabine/ cisplatin

• Completed 3 cycles of neoadjuvant gemcitabine/ cisplatin

• Underwent robotic nephroureterectomy and cystectomy. Currently under surveillance

44

Bicuspid aortic valve

• None

• Known severe aortic regurgitation with LV dilation and preserved LVEF

B-cell acute lymphoblastic leukemia

• Stem cell transplantation, thiotepa and fludarabine.

• Previously received ABFM induction including 18.75 mg/m2 of anthracycline + 2 cycles of blinatumomab.

Acute pulmonary edema resulting in hypoxic respiratory failure

10 days after stem cell transplantation, 6 months after induction

Aggressive high-dose intravenous diuretic therapy instituted

• Normalization of volume status

• Maintenance oral diuretic therapy instituted

• Continued on losartan and carvedilol

• Relapsed 6 months after stem cell transplantation

• Currently back on blinatumomab therapy

58

Pulmonary valve stenosis

• None.

• Known severe pulmonary regurgitation with normal right ventricular systolic function

Concomitant invasive ductal and lobular carcinoma in the same breast

TCH (taxotere/ carboplatin, herceptin)

• Progressive right-sided heart failure with each cycle, worst after completion of 6th and final cycle of TCH.

• No changes to ventricular systolic function on echocardiogram

21 days after 1st cycle

Oral diuretic therapy

• Normalization of volume status and resolution of heart failure

• Maintenance diuretic therapy instituted

• Underwent prophylactic right breast mastectomy.

• Currently in remission

  1. ASD Atrial septal defect, VSD Ventricular septal defect, SVT Supraventricular tachycardia, AF Atrial fibrillation, LAD Left anterior descending, LV Left ventricle, EF Ejection fraction