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septum treated by valvotomy: 95 (75%) had no shunt at the atrial level and 30 (14%) had an interatrial shunt detected preoperationally by dye-dilution curves. In 19 of the 30 patients, the atrial shunt was right-to-left and the defect was simply a patent foramen ovale; in the other 11 patients, the shunt was left-to-right and these patients had a true fossa ovalis atrial septal defect. The peak pressure gradient across the pulmonic valve was much higher in the group with a patent foramen ovale than in the group with a true atrial septal defect (120 ± 11 vs 60 ± 5mm Hg). Shunning at the atrial level in the present patient never occurred until acute tricuspid regurgitation from the valve replacement equipment in the tricuspid valve orice led to the sudden appearance of a right-to-left shunt through the previous valvular-competent patent foramen ovale.

DISCLOSURE

The authors have no conicts of interest to disclose.

REFERENCES

1. Ayad RF, Johnston SB, Grayburn PA, Schmidt TT, Choi JW. Congenital pulmonic

stenosis in a 77-year-old woman successfully treated with percutaneous balloon valvuloplasty. Proc (Bayl Univ Med Cent) 2010;23:21–23.

2. Roberts WC, Mason DT, Morrow AC, Braunwald E. Calcic pulmonic stenosis.

Circulation 1968;37:973–978.

3. Covarrubias EA, Sheikh MU, Isner JM, Gomes M, Hufnagel CA, Roberts WC.

Calcic pulmonic stenosis in adulthood. Treatment by valve replacement (porcine xenograft) with postoperative hemodynamic evaluation. Chest 1979;75:399–402.

4. Roberts WC, Vowels TJ, Filardo G, Ko JM, Mathur RP, Shirani J. Natural history

of unoperated aortic stenosis during a 50-year period of cardiac valve replacement. Am J Cardiol 2013;112:541–553.

5. Shirani J, Zafari AM, Roberts WC. Sudden death, right ventricle infarction, and

abnormal right ventricular intramural coronary arteries in isolated congenital valvular pulmonic stenosis. Am J Cardiol 1993;72:367–370.

6. Tami LF, McElderry MW. Pulmonary artery aneurysm due to severe congenital

pulmonic stenosis: Case report and literature review. Angiology 1994;45:383–390.

7. Roberts WC, Filardo G, Ko JM, Siegel RJ, Dollar AL, Ross EM, Shirani J.

Comparison of total 12-lead QRS voltage in a variety of cardiac conditions and its usefulness in predicting increased cardiac mass. Am J Cardiol 2013;112:904–909.

8. Blalock A. The surgical treatment of congenital pulmonic stenosis. Ann Surg

1946;124:879–888.

9. Coberly L, Harrison JK, Bashore TM. Percutaneous balloon pulmonic valvulo-

plasty following treated endocarditis in a patient with congenital pulmonary valve stenosis. Cathet Cardiovasc Diagn 1990;21:245–247.

10.

Roberts WC, Shemin RJ, Kent KM. Frequency and direction of interatrial shunt-

ing in valvular pulmonic stenosis with intact ventricular septum and without left ventricular inow or outow obstruction. An analysis of 127 patients treated by valvotomy. Am Heart J 1980;99:142–148.

292

Case 1697 CombineD atresia of CarDiaC VaLVes in a Premature neWborn

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Case 1697 Combined Atresia of One Left-Sided and One Right-Sided Cardiac Valve in a Premature Newborn

William C. Roberts, MD, Alan C. Sing, MD and Joseph M. Guileyardo, MD

Described herein is the heart of a 2-day-old newborn, the product of a 25-week gestation, with atresia of two cardiac valves, one on the right side and one on the left side, apparently a previously undescribed entity.

The worst heart disease—the one allowing the shortest survival—is aortic valve atresia, the most common cause of death in the rst month of life. these newborns with aortic valve atresia also have mitral valve atresia. The second most common condition associated with an atretic cardiac valve is pulmonic valve atresia. The occurrence of one right-sided atretic valve and one left-sided atretic valve in the same heart must be incredibly rare, but such was the case in the newborn described herein.

CASE DESCRIPTION

A 2-day-old female newborn after a 25-week gestation weighed 550 g. An electrocardiogram shortly after birth disclosed a prolonged P-R interval and sinus bradycardia. A technically difcult echocardiogram disclosed a dilated right ventricle, a normal-sized left ventricle, a large atrial septal defect with bidirectional ow, a large ventricular septal defect with bidirectional ow, a small (1mm) patent ductus arteriosus with left-to-right ow, severe “pulmonic stenosis,” and an unobstructed aortic valve and aortic arch. The echocardiographic ndings were interpreted as being consistent with tetralogy of Fallot.

The newborn died in the intensive care unit on the second day of life. At necropsy, the heart weighed 4.15 g. The cardiac ndings are illustrated in the Figure. Both the mitral and pulmonic valves were atretic, the atrial septum was absent, and a ventricular septal defect was located caudal to the aortic valve, which arose from the dilated right ventricle. Anarrowed patent ductus was present, and it was the only source of blood to the lungs.

In addition to the cardiac anomalies, a cleft lip was present, and it extended to involve the entire hard and soft palate. Cytogenetic SNP microarray analysis performed on an ante-mortem blood sample disclosed a normal female chromosome pattern with no deletions or duplications of known or potential clinical signicance. Postmortem chromosome analysis also showed a normal female 46 XX karyotype.

1,2

About 25% of

From the Baylor Heart and Vascular Institute (Roberts) and the Departments of Pathology (Roberts, Guileyardo) and Internal Medicine (Division of Cardiology) (Roberts), Baylor University Medical Center at Dallas; and the Department of Pediatric Cardiology, Texas Health Presbyterian Hospital, Dallas, Texas (Sing).

Corresponding author: William C. Roberts, MD, Baylor Heart and Vascular Institute, 621 N. Hall Street, Suite H-030, Dallas, TX 75226 (e-mail: William.Roberts1@bswhealth.org).

293DOI: 10.1201/9781003409342-51

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Figure Cardiac ndings of a premature newborn with combined pulmonic and mitral valve atresia. AA indicates ascending aorta; IVC, inferior vena cava; LA, left atrium; LMPA, left main pulmonary artery; LV, left ventricle; RA, right atrium; RV, right ventricle; SVC, superior vena cava.

No numerical or structural aberrations were seen at the 500 G-band resolution. Examination of the placenta disclosed a three-vessel cord, premature villous architecture, severe acute chorioamnionitis, and mild acute funisitis.

DISCUSSION

Our patient had combined pulmonic and mitral valve atresia. Such a combination, to our knowledge, has not been reported previously. Death was probably the consequence of progressive narrowing of the ductus arteriosus.

294

Case 1697 CombineD atresia of CarDiaC VaLVes in a Premature neWborn

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REFERENCES

1. Roberts WC, Perry LW, Chandra RS, Myers GE, Shapiro SR, Scott LP. Aortic valve

atresia: Anew classication based on necropsy study of 73 cases. Am J Cardiol 1976;37(5):753–756.

2. Perry LW, Scott LP III, Shapiro SR, Chandra RS, Roberts WC. Atresia of the aortic

valve with ventricular septal defect: Aclinicopathologic study of four newborns. Chest 1977;72(6):757–761.

295

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Case 1766 Huge Right Ventricular Outﬂow Tract Aneurysm Late Following Total Repair of Tetralogy ofFallot Leading to Orthotopic Heart Transplantation

William C. Roberts

1,2,3,*

and Shafﬁn Siddiqui

1,#

1. INTRODUCTION

Operative correction of patients with tetralogy of Fallot (T of F) has been one of the seminal developments in medicine in the last century. Many such patients as a consequence have had entirely normal life spans compared to patients without operative intervention whose mean span is 5years.

Despite these phenomenal operative results, some of the operative survivors develop debilitating right-sided heart failure, arrhythmias, right-ventricular outow tract (RVOT) patch aneurysms, and other forms of cardiac dysfunction. We describe herein 3 men who developed huge RVOT aneurysms, a relatively infrequently described consequence of the corrective operation for patients with T of F, and right-sided heart failure; simultaneously, 2 of them developed refractory ventricular tachycardia (VT). All 3 patients subsequently underwent orthotopic heart transplantation (OHT).

1.1. Patients Studied

Findings in the 3 men are summarized in Table1. Each had successful OHT 35, 43, and 59years, respectively, after the corrective operation at ages 6, 4, and 6years, respectively. Two developed recurring episodes of VT, one of whom had multiple ventricular ablations without preventing the VT. All 3 had evidence of right-sided heart failure, worse in the patient without VT. The RVOT patch in all 3 patients was parietal pericardium. Follow-up after OHT (11years in case #1; 7years in case #2;

Baylor Scott& White Heart and Vascular Institute,

Baylor Scott& White Health, Dallas, Texas, USA

Department of Internal Medicine (Cardiology), Baylor

Scott& White Health, Dallas, Texas, USA

Department of Pathology, Baylor University Medical Center,

Baylor Scott& White Health, Dallas, Texas, USA

Article history:

Declaration of competing interest: The authors declare that they have no known competing nancial interests or personal relationships that could have appeared to inuence the work reported in this paper.

* Corresponding author.

E-mail address: william.roberts1@bswhealth.org (W.C. Rober ts).

Shafn Siddiqui, Junior, Princeton University, Princeton, New Jersey.

296

DOI: 10.1201/9781003409342-52

Case 1766 huge right VentriCuLar outfLoW traCt aneurYsm

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and 5 months in case #3) disclosed that all 3 patients were asymptomatic and quite active.

Table 1: Comparison of ndings in the 3 men having cardiac transplantation

decades after total repair of tetralogy of Fallot.

Variable Case 1

Age (years)

At repair of tetralogy of Fallot 6 4 6 When RV outow tract aneurysm rst noted 26 33 60 At onset of heart failure 26 33 60 At onset of ventricular tachycardia 26 33 No VT At orthotopic heart transplantation 41 47 65 At January2021) Patch=Parietal pericardium + + + RV outow patch calcied +++ + +++ Body mass index (kg/m2) Bundle branch block +(Left) +(Right) +(Right) Total 12-lead QRS voltage (mm) 156 71 84 Intracardiac debrillator (age) +(36) +(33) +(61) Radiofrequency ablation + 0 0 Pre-OHT Hemodynamics

Left ventricle (s/d) (mm Hg) 95/30 Pulmonary artery (s/d) (mm Hg) 35/15 45/20 45/5 Right ventricle (s/d) (mm Hg) 45/20 45/10 45/5 Lowest LV ejection fraction (%) 10 50 20 Heart weight (g) 655 505 590 Cardiac adiposity (oating heart) + + + Coronary artery disease 0 0 0

LV= left ventricular; RV=right ventricular; s/d=peak systolic/end diastolic; OHT=orthotropic heart transplant.

From George BA, Jong MK, Lensing FD, Kuiper JJ, Roberts WC. “Repaired” tetralogy of Fallot mimicking arrhythmogenic right ventricular cardiomyopathy. Am J Cardiol 2011;108:326–329.

From Donaldson EE, Jong MK, Gonzales-Stawinski G, Hall SA, Roberts WC. Secondary arrhythmogenic right ventricular cardiomyopathy decades after operative repair of tetralogy of Fallot. Am J Cardiol 2014;114;806–809.

****

Case 2**Case 3

51 54 65

29 →25(OHT) →33

32 29

— —

297

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2. DISCUSSION

Each of the 3 patients described developed huge RVOT aneurysms involving the transannular patches decades after the operative correction of T of F. Calcic deposits developed in the parietal pericardial patches of all 3 patients, minimal in one and massive in 2. Conrmation of the presence of calcic deposits in the patches has been infrequent. tively early after its insertion. Seybold-Epting etal.

2–7

Calcic deposits in the patch can develop rela-

reported 10 patients among 252 patients with corrected T of F who had a parietal pericardium RVOT patch inserted, and 4 of them were conrmed to have developed microscopic-sized patch calcic deposits 6 to 36 months after the operation. This time span is too short to recognize patch calcic deposits by imaging studies or grossly. In contrast, the patients of this study accumulated the calcic deposits over the course of 35, 43, and 59years. Ross etal.

reported a 16-year-old boy who developed massive calcic deposits in a parietal pericardial RVOT patch within 7 years after its insertion.

Two of the 3 patients described had recurring VT, uncontrolled by either multiple antiarrhythmic agents or repeated ablation procedures. One of the 2 patients with VT had classic epsilon waves on the electrocardiogram, characteristic of arrhythmogenic right ventricular cardiomyopathy.

The occurrence of OHT after repair of T of F is an uncommon event (Table2). Each of the 3 patients described herein returned to normal activities after their OHT. All 3 are asymptomatic as of January2021.

A previously unreported nding in patients with repaired T of F is excessive cardiac adipose tissue. The hearts in all 3 patients described herein oated in a container of formaldehyde indicating excessive cardiac adipose tissue (adipose tissue is lighter than myocardium). The fat surrounding the left ventricle was especially extensive, an unusual feature. All 3 patients were overweight.

This manuscript, of course, has limitations: 1) Only 3 cases are described but OHT is, as Table2 demonstrates, uncommon after total “correction” of T of F. Few other studies, however, have had such long intervals between the corrective operation and the OHT. 2) Although all 3 patients had huge RVOT aneurysms and right-sided heart failure, 2 patients had recurring VT prior to OHT (Figures1–3).

298

Table 2: Previous publications of patients having repair for tetralogy of Fallot (T of F) with insertion of right ventricular outow tract

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(RVOT) patch with follow-up thereafter.

First author (reference)

Publication

year

Patients

with

T of F

Patients

receiving

RVOT

patch

Material of

patch

Age (years)

at total

repair of T

of F range

(mean ± SD)

Duration of

follow-up

(years)range

(mean ± SD)

[median]

Patients

developing

patch

aneurysm

[median]

Payne

Rosenthal Kaplan

Rieker Chiariello Seybold-Epting31977 252 252 Pericardial — 0.5–3 10 — — Fuster

Kirklin Nollert

Knot-Craig Nørgaard

Gatzoulis

299

1961 58 26 Ivalon — 0.75–4 (1.9) 3 — —

6 Ivalon -Teon — 0.66–2 (1.3) 0 — —

26 Teon — 0.5–1.5 (0.6) 0 — — 1972 — 135 Pericardial — — (0.5) 8 — — 1973 150 54 Pericardial 0–26 0.5–14 16 — —

13 Homograft 0–26 0.5–14 1 — — 1975 60 Teon 4–20 — 0 — — 1975 403 144 Pericardial 0.25–41 — (1.25) 7 — 0

1980 396 — Ivalon or

Teon 1989 791 255 Dacron — [5] >2–20 (9 ± 5) 2 — 0 1993 490 — — — (12 ± 9) 1.06–35 (25 ±

1997 193 — — — (2 ± 0.2) 0.1–26 (11 ± 7) — — — 1999 109 60 Dacron or

Pericardial 2000 793 274 274 — (8 ± 8) [6] 8–41 (21 ± 9) — 33 0

0–54 12–22 3 1 0

— 0 0

13)

— [13] 20–28 (26) — — 1

Patients

developing

Patients

having

OHT

Case 1766 huge right VentriCuLar outfLoW traCt aneurYsm

300

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Troost Munkhammar

Kotani

Cuypers Galicia-Tornell Dłuzniewska

Simon

Castilhos Padalino

2007 68 — — — [24] 1.3–18 (— ± 8) — — 3

2013 31 — — — [0.8] 2–16 (9 ± 3) — 0 0 2013 41 11 Dacron 0.5–3.5 (0.9) — [5.9] — 0 0 2014 80 48 — 1.7–6.6 (4.3) 31–43 — 26 0

2015 52 52 Dacron — (4 ± 2) — (6 ± 2) 0 0 0

2018 83 54 — 0–30 [3] — (30) — 13 0 2019 38 27 — — (3.3 ± 1) 19.5–35.8

2019 206 65 — 2–4 [3] — (21 ± 8.2) — 15 0 2019 720 435 Pericardial or

TOTALS 5014 1937

OHT=orthotopic heart transplant; VT=ventricular tachycardia; — = no information available.

These percentages are out of the number of patients with RVOT patch (1,937 patients).

(2.58%)

homograft

— 15 —

(30.9)

3.7–11.7 (5.7) 1–21 — — 0

50 (0.03%)

103 (0.06%)

4 (0.002%)

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Case 1766 huge right VentriCuLar outfLoW traCt aneurYsm

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Figure 1 Case #1. The heart. (a) Outside of the heart showing a huge quantity of adipose tissue covering both ventricles. (b) Opened right ventricle showing the huge aneurysm in the outow tract. (c) Anterior wall of the left ventricle showing a healed ablation site, consisting of brous tissue and adipose tissue. The coronary arteries are free of plaque. The ablation had been performed 20years earlier. LAD=left anterior descending; LV=left ventricular cavity; RV=right ventricular cavity.

Figure 2 Case #2. Opened right ventricle showing the huge aneurysm in the outow tract. Both ventricles are nearly covered by adipose tissue. The coronary arteries are wide open.

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