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Presentation & Chief Complaint

This is a 63 yo female who presents to OSH with:“Altered Mental Status”

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Outside Hospital Course• Admission labs notable for coagulopathy c/w end-stage liver

disease, lactic acidosis and elevated ammonia

• Started on broad-spectrum antibiotics and lactulose+rifaximin

• EGD with gastric ulcers and EVs, no active bleeding

• Progressively worsening renal dysfunction, unclear etiology but thought d/t elevated intraabdominal pressures

• Failed multiple attempts to extubate

• Transferred to UCSD for urgent liver transplant evaluation

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Arrival to UCSD

• Intubated, SpO2 98% on VTPC FiO2 40%

• Sedated on fentanyl and Propofol gtt

• Levophed pressor support

• Ceftriaxone IV for SBP ppx

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Physical Exam• Vitals: Afebrile, BP 96/54, HR 67, SpO2 97% w/ ETT (FiO2

40%)

• General: intubated, sedated, jaundiced• HEENT: perrl, MMM, icteric sclera• NODES: no cervical, supraclavicular LNs palpable• CV: Normal rate and regular rhythm, no murmurs, no extra

heart sounds• Chest: coarse breath sounds bilaterally on anterior/lateral

exam• Abdomen: tense ascites, no TTP• Extremities: no clubbing, 2+ edema LLE, trace RLE edema

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TTE Report

• LVEF 73%• Normal LV size and function• Normal RV size and function• Mild pHTN (RVSP 42 mmHg)

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TTE Report

• Contrast injection reveals sonolucent circular structure in interatrial septum consistent with the diagnosis of bronchogenic cyst

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CT Thorax Report

• Indeterminate low attenuating/cystic mass centered near the interatrial septum

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Hospital Day 4:MRI W and WO Contrast

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T2

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Delayed

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Cardiac MRI Report• Findings:

– There is a 22 x 23 mm lesion noted in the interatrial septum, in the expected region of the AV node. The mass demonstrates fluid signal on T1 and T2 weighted imaging. No fat is identified within the lesion. The lesion demonstrates no perfusion or internal enhancement.

– Limited evaluation of the remainder of the heart demonstrates normal myocardial morphology with no myocardial delayed enhancement.

• Impression:– 23 mm benign-appearing cystic lesion in the interatrial septum,

in the expected location of the AV node, likely representing a benign lesion such as a cystic tumor of the AV node

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Cystic Masses of the Interatrial Septum

• AV Nodal Cysts• Bronchogenic Cysts

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AVN Cysts• ~70 published case reports since first known case was published in

1911, the majority discovered incidentally on autopsy

• Thought to be mesothelioma of AV node, although endodermal v. mesothelial origin of the tumor remains controversial (tumors were previously referred to as “AVN mesothelioma”)

• Majority of cases are thought to be benign

• Female predominance, cases published in newborns to 86 years old, mean age 38 years

• Can grow in size and cause variable conduction block, syncope or even sudden death

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A “benign” tumor?

• “The smallest tumor which causes sudden death.” Wolff PL et al. JAMA 1965

• “Fatal electrical instability of the heart associated with benign congenital polycystic tumor of the atrioventricular node.” James TN et al. Circulation 1977

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1107

Intracardiac bronchogenic cyst is a rare congenital malfor-mation that is a remnant from abnormal budding of the

embryonic foregut. Among the intracardiac cases, broncho-genic cysts of the interatrial septum account for three-fourths of the reported cases.1 Because most of the intracardiac cysts are surgically removed promptly after detection, the natural course of intracardiac bronchogenic cyst is unknown. Here, we report the case of a growing interatrial bronchogenic cyst incidentally detected during the postoperative surveillance of gastric cancer.

Case PresentationA 35-year-old male patient was referred to the cardiology department for an incidentally detected intracardiac mass. The patient had been diagnosed with advanced gastric cancer 14 months before referral, had undergone total gastrectomy, and was receiving adjuvant chemotherapy. The mass was first noted by his surveillance abdominal computed tomography (CT) scan, which covered his heart. Retrospective review of his preoperative stomach CT scan and follow-up abdomen CT scan revealed that the cyst had increased up to 6-fold in vol-ume over a period of 13 months (Figure 1 and Figure I in the online-only Data Supplement).

At the time of referral, the patient was asymptomatic. Six years ago, he had an excision of a left second branchial cleft cyst. His medical history was otherwise unremarkable. The results of physical examination, chest radiograph, and routine blood sample analysis were normal. Twelve-lead ECG showed sinus rhythm with first-degree atrioventricular block (Figure IIA in the online-only Data Supplement). Transthoracic echo-cardiograms revealed a 2.1×2.1 cm cystic mass in the right atrial side of the interatrial septum. Contrast echocardiography with agitated saline revealed that the cyst had no flow commu-nication with right atrium and ventricle (Figure 2, Movie I in the online-only Data Supplement). Other echocardiographic findings were normal. The estimated pressure of each atrium was 2 to 6 mm Hg for the right atrium and 4 to 12 mm Hg for the left atrium, respectively. ECG-gated CT angiography showed a cystic mass without contrast enhancement. Cardiac magnetic resonance image revealed high signal intensity on

a fat-suppressed black blood T2-weighted image, low sig-nal on black blood T1-weighted image, and no enhancement (Figure 2). From these findings, the cyst was assumed to have benign nature. However, because the mass was rapidly grow-ing, based on CT scans of 5-month intervals, malignancy could not be completely excluded. Because the patient was undergoing his second-line adjuvant chemotherapy, the opera-tion was deferred until the completion of chemotherapy.

Two months after his last chemotherapy cycle, the patient was admitted for an operation. Anterior right minithoracot-omy and right atriotomy under cardiopulmonary bypass was successfully performed. An ≈3×2 cm round unilocular gray-white cyst was noted and completely excised. The cyst con-tained amber-colored mucous fluid. The histological analysis of the specimen revealed a benign cyst lined by pseudostrati-fied cuboidal and columnar cells with focal cilia. Based on the pathological findings, the patient was diagnosed as having an interatrial bronchogenic cyst (Figure 3)

At immediate postoperation, 12-lead ECG showed com-plete atrioventricular block, which persisted 6 days after surgery. A dual-chamber pacemaker was then implanted before discharge (Figure IIB and IIC in the online-only Data Supplement). There was no other postoperative complication. At 2 months follow-up, the patient was symptom free without any evidence of cardiac tumor or gastric cancer recurrence.

DiscussionHere, we report a case of incidentally detected intracardiac bronchogenic cyst that was rapidly growing. Regarding the growth of an intracardiac bronchogenic cyst, there is only 1 case report of a 68-year-old woman who initially refused sur-gery at diagnosis. The interatrial bronchogenic cyst remained asymptomatic for 2 years without significant change in the size of the cyst.2 To our knowledge, this is the first report doc-umenting the growth of an intracardiac bronchogenic cyst in an adult. In our case, the volume of cyst increased >6-fold in 13 months. The cyst was rather negligible for 34 years, but grew to a 3×2 cm cyst for 1 year. In general, the growth of bronchogenic cyst is thought to be the result of mucus accu-mulation, bleeding, infection, or malignant transformation.

(Circulation. 2014;130:1107-1109.)© 2014 American Heart Association, Inc.

Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIRCULATIONAHA.114.010992

From the Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea (J.P.); Department of Internal Medicine (G.-Y.C., I.-Y.O) and Department of Thoracic and Cardiovascular Surgery (K.-H.P.), Seoul National University Bundang Hospital, Gumi-dong, Bundang, Seongnam, Gyeonggi-do, South Korea.

The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.114. 010992/-/DC1.

Correspondence to Il-Young Oh, MD, Assistant Professor, Cardiovascular Center, Seoul National University Bundang Hospital, 82 Gumiro-173-gil, Bundang, Seongnam, Gyeonggi, 463-707, South Korea. Email ilyoung.oh@snubh.org

Intracardiac Bronchogenic CystReport of a Growing Lesion

Jonghanne Park, MD; Goo-Yeong Cho, MD, PhD; Kay-Hyun Park MD, PhD; Il-Young Oh, MD

Images in Cardiovascular Medicine

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2014

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1108 Circulation September 23, 2014

Recent study of antenatally diagnosed bronchogenic cysts revealed that the cysts grow slowly in the first months of life but grow exponentially even in the absence of complication.3 The clinical presentation, image findings, and gross findings of the cyst in this case excluded hemorrhagic and infectious causes. Histopathologic examination of the cyst showed no evidence of malignant transformation. Therefore, we specu-lated that chronic inflammation precipitated by adjuvant che-motherapy could be the cause of cyst growth. The patient received oxaliplatin for 3 months and oral 5-fluorouracil prodrugs, capecitabine followed by S-1, for 1 year. The cyst grew 6-fold during that period. Mucositis and diarrhea are well-known adverse effects of 5-fluorouracil. Recent studies have revealed that the proinflammatory process and decreased reabsorption of water is the possible underlying mechanism of 5-fluorouracil–induced mucositis and diarrhea.4 Interestingly, inflammatory cell infiltration with multiple lymphocyte aggre-gates was found at the submucosal layer of the cyst (Figure III in the online-only Data Supplement). Chronic inflammation attributable to chemotherapy might have resulted in increased mucus accumulation and cyst growth.

With the development of imaging modalities, >50% of bronchogenic or benign cysts are diagnosed preoperatively. However, surgical removal of the lesion remains the mainstay of therapy. For intracardiac cysts, there is not a report of expectant management even in an asymptomatic patient. Although there

is a chance that only surgically confirmed cases are reported in the literature, it is likely that the concern of malignant trans-formation and complication (ie, hemodynamic compromise, conduction disturbance, or embolism of cyst material) may urge surgical excision. The rare incidence and prompt surgical excision of intracardiac bronchogenic cysts limit the chance of observing serial growth of the benign lesion.

AcknowledgmentWe thank Jin-Haeng Chung, MD, PhD and Yoon Jin Kwak, MD for their pathological review and discussion.

DisclosuresNone.

References 1. Vaideeswar P, Agnihotri MA, Patwardhan AM. Intracardiac bronchogenic

cyst. J Card Surg. 2011;26:266–268. 2. Lee T, Tsai IC, Tsai WL, Jan YJ, Lee CH. Bronchogenic cyst in the left

atrium combined with persistent left superior vena cava: the first case in the literature. AJR Am J Roentgenol. 2005;185:116–119.

3. Maurin S, Hery G, Bourliere B, Potier A, Guys JM, Lagausie PD. Bronchogenic cyst: clinical course from antenatal diagnosis to postnatal thoracoscopic resection. J Minim Access Surg. 2013;9:25–28.

4. Sakai H, Sagara A, Matsumoto K, Hasegawa S, Sato K, Nishizaki M, Shoji T, Horie S, Nakagawa T, Tokuyama S, Narita M. 5-Fluorouracil induces diarrhea with changes in the expression of inflammatory cytokines and aquaporins in mouse intestines. PLoS One. 2013;8:e54788.

Figure 1. Rapidly growing intracardiac mass inci-dentally detected during abdomen CT follow-up. Top left, At diagnosis of gastric cancer (13 months before referral). Top right, 7-month follow-up (6 months before referral). Bottom left, 12-month follow-up (1 month before referral). Bottom right, 13-month CT angiography (at referral). Scale bar, 1 cm. CT indicates computed tomography.

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Initial CT Scan 7 months

12 months 13 months

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Park et al Growth of Intracardiac Bronchogenic Cyst 1109

Figure 2. Multimodality characterization of inter-atrial cyst. Top row, 2.1×2.0 cm low echogenic cyst without blood flow communication on contrast echocardiography. Middle row, ECG-gated CT angiography of interatrial cyst shows absence of postcontrast enhancement. Bottom row: Left, Fat-suppressed black blood T2-weighted image; Middle, black blood T1-weighted image; Right, gadolinium-enhanced black blood T1-weighted image. CT indicates computed tomography.

Figure 3. Surgical resection of cyst and histopathologic diagnosis of bronchogenic cyst. Cyst lined by pseudostratified cuboidal and columnar cells with focal cilia (hematoxylin-eosin stain, ×400).

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Contrast Echo

CT Thorax

cMRI

T2 T1 Gad-enhanced T1

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Park et al Growth of Intracardiac Bronchogenic Cyst 1109

Figure 2. Multimodality characterization of inter-atrial cyst. Top row, 2.1×2.0 cm low echogenic cyst without blood flow communication on contrast echocardiography. Middle row, ECG-gated CT angiography of interatrial cyst shows absence of postcontrast enhancement. Bottom row: Left, Fat-suppressed black blood T2-weighted image; Middle, black blood T1-weighted image; Right, gadolinium-enhanced black blood T1-weighted image. CT indicates computed tomography.

Figure 3. Surgical resection of cyst and histopathologic diagnosis of bronchogenic cyst. Cyst lined by pseudostratified cuboidal and columnar cells with focal cilia (hematoxylin-eosin stain, ×400).

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Bronchogenic Cysts• Intracardiac Bronchogenic cyst: rare congenital

malformation caused by a remnant from the abnormal budding of the embryonic foregut

• 3/4 cases of intracardiac bronchogenic cyst occur in the interatrial septum

• Most descried bronchogenic cysts were surgically removed promptly after detection, so the exact natural course is unknown

• Similarly to AVN cysts they can rapidly enlarge, cause heart block and sudden death

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Hospital Day 5:Liver Transplant Recs

• Declined to list for transplant due to comorbidities– Invasive aspergillosis on BAL– Hemodynamic Instability– Left ovarian cyst found on CT A/P– Splenosis on CT A/P– Interatrial Cyst

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Hospital Day 8

• Ongoing GOC discussions with family given poor prognosis

• Family decided to transition to DNAR with full care

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Hospital Day 9-10

• Patient condition continued to deteriorate with escalating pressor requirement and ongoing AfibRVR

• Decision made to transition to comfort care and withdraw care

• Hospital Day 10: patient expired

• Family declined autopsy

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